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Full-thickness macular hole is a rare complication of macular telangiectasia type 2, and its treatment is still controversial. A patient with a full-thickness macular hole secondary to macular telangiectasia type 2 underwent vitreoretinal surgery with a plasma rich in growth factors membrane in the macular hole. At the sixth month of follow-up, anatomical and functional improvements were achieved, with no adverse effects. Plasma rich in growth factors is a new option, with advantages due to its biological properties that achieves good results in terms of safety and effectiveness in the surgical treatment of macular hole.

Idiopathic macular telangiectasia type 2 (MacTel 2) is a slow and progressive bilateral condition that affects middle-aged and elderly individuals. Vision loss is generally mild and occurs over the course of many years. The development of sub-retinal neovascularisation (SRNV) can occur late in the disease process, and lead to more dramatic vision loss. A report is presented of 2cases of MacTel 2 in which optical coherence tomography angiography (OCTA) was essential for the diagnosis of secondary SRNV. The commercially available OCTA Cirrus AngioPlex 5000 platform (Zeiss, Jena, Germany) was used. Subretinal neovascularization was detectable in both cases in OCTA at the level of the deep capillary plexus and the avascular layer. OCTA also allowed us to monitor disease progression and monitor response to anti-VEGF therapy.

We present a case report of an autologous internal limiting membrane (ILM) graft transposition technique in a 60-years-old female patient who with a full-thickness macular hole (FTMH) secondary to idiopathic macular telangiectasia type 2 (MacTel type 2). To our knowledge, our case is the first report of ILM graft transposition to treat persistent FTMH secondary to MacTel type 2. MacTel type 2 is an acquired disease characterized by macular capillary abnormalities and retinal neurodegeneration. FTMH is an infrequent complication of MacTel type 2. Pars plana vitrectomy (PPV) with ILM peeling and gas endotamponade is the standard treatment of FTMH to help restore macular anatomy and visual function. The technique of autologous ILM graft transposition is a useful alternative to repair large, persistent macular holes that have failed to close with previous ILM peeling. In the present case, our patient with persistent FTMH secondary to MacTel type 2 after the previous standard macular hole surgery was admitted to our clinic. We performed 23-gauge PPV with extramacular ILM peeling and ILM graft transposition into the macular hole. 2 months after the surgery, her visual acuity had improved and anatomic closure of the FTMH was observed. In our opinion, ILM grafting might support reorganization of the retinal structure that has been affected by Muller cell degeneration.

Idiopathic macular telangiectasia type 1 (MacTel 1) almost always occurs unilaterally. In this article, we report the case of a 73-year-old man with no significant history diagnosed with MacTel 1 but with atypical bilateral presentation, something very rare in this disease. The usefulness of multimodal imaging studies, including optical coherence tomography angiography (OCTA), is highlighted to differentiate it from macular telangectasia type 2 (MacTel 2). The patient's condition was characterized by cystoid macular edema (CME) with discreet results with treatment with a dexamethasone implant. However, aflibercept therapy showed favorable results, but with recurrences when extending the doses.

Purpose: To evaluate long-term visual prognosis and changes in the retinal microstructure in patients with macular telangiectasia type 2.Methods: Retrospective analysis of medical records were performed for 61 eyes (32 patients) diagnosed with macular telangiectasia type 2. The visual acuity at diagnosis was compared with that at the final visit. In addition, cases were classified into three grades based on optical coherence tomography (OCT) findings: grade 1, inner retinal cavities only; grade 2, outer retinal cavities with ellipsoid zone disruption; and grade 3, ellipsoid zone disruption with proliferation of retinal pigment epithelium. Change in the grade during the follow-up was verified. In addition, visual acuities were compared between eyes with and without a change in grade.Results: The mean follow-up period was 41.5 ± 33.2 months. Visual acuity had deteriorated significantly from the mean logarithm of the minimal angle of resolution 0.23 ± 0.26 at diagnosis to a mean of 0.31 ± 0.31 at the final visit (p < 0.001). When classified using OCT, 65.6%, 22.9%, and 11.5% were grades 1, 2, and 3, respectively, at diagnosis and 47.5%, 36.1%, and 16.4% at the final visit. Progression of grade was noted in 22.9%. A higher degree of visual deterioration was noted in eyes showing progression than in eyes without (p = 0.002).Conclusions: During the long-term follow-up period, visual deterioration was noted in patients with macular telangiectasia type 2. However, the degree of deterioration was not high. Progression in the disruption of the retinal microstructure was the likely cause of visual deterioration.

We investigated the structural findings on spectral-domain optical coherence tomography (SD-OCT) related to the presence of right-angled vessels (RAV) in patients with macular telangiectasia (MacTel) type 2 with severity 3 in Korea. A retrospective multicenter cross-sectional study was conducted in six tertiary hospitals in Korea; the study included 116 MacTel type 2 eyes with severity 3. The SD-OCT findings were compared between eyes with RAV on fundus photography or fluorescein angiography and those without RAV. Logistic regression was performed to determine factors associated with the presence of RAV. Fifty eyes presented with RAV and 61 eyes without RAV. More eyes presented with only inner retinal (IR) cavities on SD-OCT among eyes without RAV than among those with RAV (P P

Macular telangiectasia type 2 (MacTel) is bilateral disease with characteristic alterations of the macular capillary network along with decreased macular pigment in the parafoveal area. The purpose of this study was to highlight that some eyes show microvascular changes which precede any visible neuronal changes on spectral-domain optical coherence tomography (SD-OCT). This observational study was conducted at a tertiary eye institute. From a registry of 630 patients with MacTel, we identified 4 patients with typical MacTel characteristics in only one eye with no visible changes on colour photographs or SD-OCT in the fellow eye. These 4 patients had findings of MacTel documented by colour fundus photograph, multicolour imaging (MCI), fundus autofluorescence (FAF), confocal blue reflectance (CBR), SD-OCT, and OCT-Angiography (OCT-A) in one eye. OCT-A was performed in MacTel patients using the High-resolution Spectralis (Heidelberg Engineering) module with a full-spectrum probabilistic approach and we employed a 30° x 15° (~8.8 mm × 4.4 mm) scan pattern covering the macula. MCI was done at the end so as to avoid fading the confocal blue reflectance hyperreflectivity seen in MacTel. On OCT-A, all 4 fellow eyes showed telangiectasia and foveal avascular zone changes in the superficial and deep capillary plexuses with no changes on SD-OCT. None of the eyes showed typically increased reflectance on CBR around the foveal area. These findings show that microvascular changes on OCT-A may occur before visible neurodegenerative changes on OCT, providing new insights into the course of the disease.

PURPOSE To present a case of macular hole associated with macular telangiectasia Type 2 (Mac-Tel 2) which underwent spontaneous closure. METHODS A 58-year-old woman is admitted with complaints of blurred and distorted vision. Optical coherence tomography, fundus autofluorescence, and optical coherence tomography angiography were performed for diagnosis and follow-up. RESULTS Optical coherence tomography revealed hyporeflective cavitations in both eyes, and full-thickness macular hole covered with internal limiting membrane drape in the left eye. Fundus autofluorescence showed loss of hypoautofluorescence in temporal parafoveal area in the right eye, and centrally in the left one. Vascular irregularities were detected in optical coherence tomography angiography images. The patient was diagnosed as Mac-Tel 2. During the follow-up, the macular hole underwent a process of spontaneous closure, starting as bridging at the level of external limiting membrane and outer nuclear layer followed by further repairment and organization of the outer plexiform layer, accompanied with visual gain. CONCLUSION Spontaneous closure of macular hole in patients with Mac-Tel 2 is a rare entity. We hypothesize that extension or proliferation of Muller cells, which leads to reconstruction of normal foveal structure, is the main mechanism for this phenomenon.

AimsTo identify morphological characteristics preceding the development of exudative neovascularisation secondary to Macular Telangiectasia type 2 (MacTel) using multimodal retinal imaging.MethodsIn this retrospective study, eyes with a minimum observation period of 6 months prior to the de novo diagnosis of an exudative neovascularisation secondary to MacTel were analysed. Morphological changes preceding the formation of neovascularisation were evaluated using colour fundus photography, infrared imaging, fluorescein angiography, macular pigment measurement and optical coherence tomography (OCT). OCT-angiography (OCT-A) images were additionally available in a subset of patients.ResultsTwenty eyes from 20 patients were examined over a median period of 17 months (range: 6–100 months). Eyes were characterised by an accelerated progression of ellipsoid zone loss (median of 0.013 mm2/month), increased thickness of the temporal parafovea and hyper-reflective lesions on OCT. The latter underwent morphological changes preceding the development of exudative neovascularisation, including an increase in size and density, and expansion to outer retinal layers and the retinal pigment epithelium. All eyes showed a foveal depletion of macular pigment. On OCT-A, a focal increase in blood flow was observed at the level of the outer retina/choriocapillaris, and retinal–retinal and retinal–choroidal anastomoses preceded the formation of exudative neovascularisation.ConclusionsMultimodal imaging allows the identification of prognostic morphological features preceding the formation of exudative neovascularisation in MacTel. Eyes exhibiting these characteristics should be monitored closely and patients should be alert for emergent symptoms in order to detect and treat neovascularisation early and, thereby, prevent irreversible visual loss.

Purpose To report a spectral-domain (SD) OCT clinical sign, outer foveal microdefect (OFMD), corresponding to a focal disruption of the foveal photoreceptors in association with various macular conditions. Design Retrospective cohort study. Participants Forty-five patients with OFMD. Methods All patients were imaged with color photography and SD OCT, and some were imaged with autofluorescence imaging, fluorescein angiography, indocyanine green angiography, or a combination thereof. Patient demographics, OFMD imaging, courses, and outcomes were analyzed retrospectively. Main Outcome Measures Demographics, conditions associated with OFMD, and diameter of OFMD. Results Fifty-one eyes of 45 patients (15 men and 30 women; age range, 10–88 years) were included. Symptoms included central scotoma, metamorphopsia, and mild to moderate visual loss. Outer foveal microdefects were observed in association with various macular conditions: presumed posterior vitreous detachment, vitreomacular interface changes, or both (24 eyes); sequelae of macular edema (3 eyes); blunt trauma (2 eyes); retinal phototoxicity resulting from laser or solar maculopathy (5 eyes); and macular telangiectasia type 2 (2 eyes). An improvement with a reduction in OFMD diameter was documented in 9 of 14 eyes that could be followed up. Conclusions We suggest renaming the condition outer foveal microdefect instead of macular microhole, which is usually associated with a vitreomacular disorder. The pattern indeed also was observed in traumatic or degenerative conditions. We assumed that various injuries led to changes in foveal morphologic features and that a full recovery of the foveal architecture could be altered, especially in eyes with findings suggestive of the pachychoroid spectrum of disorders.

Purpose : To evaluate the long-term natural course of macular telangiectasia Type 2, correlation with visual acuity and the incidence of Choroidal Neovascularisation (CNV) in Indian eyes. Material and methods : A Retrospective analysis of Patients with MacTel Type 2 was done over a period of 12 years with all patients having a minimum of 3 years follow up. The demographic details and ocular characteristics including best-corrected visual acuity (BCVA), fundus photography, fluorescein angiography, and optical coherence tomography images were studied in both proliferative and non-proliferative MacTel. Mixed models were used to estimate progression rates and a Kaplan Meier estimation of BCVA was plotted. Results : Eighty-two eyes of 47 patients were studied over a period of mean duration of 4.5 years (range: 3 years-8.5 years). There was no difference in the demographic characteristics between the non-proliferative MacTel and proliferative MacTel groups. There were no significant risk factors observed for progression. However, patients with retinal greying had significant risk reduction for a BCVA decline. The mean logMAR BCVA decreased from 0.25 ± 0.25 at baseline to 0.46 ± 0.42 by 4 years. Twenty-eight percent of the patients maintained their vision 8 years from baseline and were unlikely to progress. The incidence of CNV was 10.6% and the mean duration for the development of CNV was 2.36 years from baseline. Seventy-Three percent (11 of 15) patients with CNV had a BCVA of Conclusion : In patients of MacTel, the maximum vision loss occurred at the fourth year and then stabilized. The major cause of poor vision observed was CNV (active in 10.98% and scarred in 7.32%), foveal atrophy (10.98%) and central pigmented plaques (3.66%). The incidence of sight-threatening complication of CNV (10.6%) is likely to occur only in a minority of eyes.

This case report describes a simple hemorrhage (SH) presenting as radial hemorrhage in Henle’s fiber layer (HFL) in a patient with high myopia. A 26-year-old girl with high myopia was referred to our center for sudden onset of decreased vision and a central scotoma in the right eye (OD). Best corrected visual acuity (BCVA) was 20/100 OD. Fundus examination showed a stellate intraretinal hemorrhage in the fovea of the OD. The hemorrhage was organized in a peculiar petaloid pattern with feathery distal edges, suggesting localization within the radially oriented HFL. The presence of both choroidal neovascularization and microvascular abnormalities consistent with macular telangiectasia type 2 (MacTel 2) were excluded. Based on these findings, a diagnosis of myopic SH was made. At 4-month follow-up BCVA OD spontaneously improved to 20/40, without any treatment been ever administered to the patient. Spectral-domain optical coherence tomography OD showed reabsorption of the hemorrhage and almost complete restoration of the foveal architecture. The intraretinal location and spread of the hemorrhage into the HFL in our patient are an unusual presentation of SH, which vividly highlights the anatomy of the fovea. Since fibers in HFL are quite delicate and loosely arranged, this layer is very susceptible to deposition of transudates, exudates, hemorrhage, and other products. Radial hemorrhage in HFL has been originally reported in 4 patients as complication of MacTel 2. It has been previously postulated that it may represent a characteristic finding in MacTel 2 that may develop as a result of microvascular abnormalities of the deep retinal capillary plexus. On the contrary, our data suggest that radial hemorrhage in the HFL does not represent a characteristic finding of MacTel 2, but must rather be considered a non-specific sign with multiple possible etiologies.

We report a bilateral case of type 1 idiopathic macular telangiectasia (IMT) in a female patient. A 40-year-old otherwise healthy female with gradual vision loss since 2 years ago with best-corrected visual acuity of 20/400 in both eyes was referred. Her past medical history was negative for any systemic disease including diabetes and systemic hypertension. Color fundus photography showed bilateral loss of normal foveal reflex with macular edema. Fluorescein angiography demonstrated symmetric perifoveal telangiectasia mainly in the superior and nasal macula in both eyes with late staining and leakage. Spectral-domain optical coherence tomography revealed significant intraretinal fluid bilaterally and subretinal fluid in the left eye. Optical coherence tomography angiography (OCTA) revealed obvious saccular parafoveal capillary telangiectasia and capillary dropout as well as decreased vascular density in both superficial and deep capillary plexus. Deep capillary plexus involvement in OCTA was more evident than superficial plexus. Based on the patient’s medical history and multimodal imaging, the diagnosis of bilateral IMT type 1 was made. The patient underwent 5 intravitreal monthly injection of bevacizumab in both eyes, which resulted in macular edema resolution. However, after 3 months of discontinuation of intravitreal bevacizumab, macular edema relapsed. In conclusion, type 1 IMT can occur bilaterally in an otherwise healthy female patient as a very rare presentation. To the best of our knowledge, this case is the 4th case of bilateral type 1 IMT reported in a female.

Purpose: To report a case of macular telangiectasia (MacTel) showing the features of type 3 disease in one eye and type 2 disease in the other eye. Methods: Descriptive case report Case description: A 50-year-old well-controlled diabetic man presented with complaints of distorted vision in the right eye for the past 1 year. His best-corrected visual acuity in both eyes was 20/20, N6. His anterior segment findings were normal. The right eye fundus showed an irregular perifoveal greying and a localised dark patch temporally inside the perifoveal region. Few microaneurysms were seen on clinical examination. No hard exudates were noted. The left eye fundus showed a few microaneurysms with minimal perifoveal greying temporally. Imaging with confocal blue reflectance, fluorescein and optical coherence tomography angiography confirmed the diagnosis of MacTel type 3 in the right eye and type 2A stage 2 in the left eye with mild non-proliferative diabetic retinopathy in both eyes. Conclusion: This case raises the possibility that type 3 disease could be a progressive non-proliferative form of a pre-existing type 2 MacTel disease.

Purpose This study aimed to demonstrate the clinical course of Japanese patients with macular telangiectasia type 2 (MacTel-2). Methods This retrospective observational case series included 16 eyes of 8 Japanese patients (3 men and 5 women) with MacTel-2. The mean age and follow-up duration was 66.9 years and 42.8 months, respectively. Differences in best-corrected visual acuity (BCVA), funduscopic macular findings, central macular thickness (CMT), and the length of macular ellipsoid zone (EZ) loss were compared between the initial/baseline and final visits. Optical coherence tomographic changes in CMT by >= 20% and in EZ loss by >= 20% or >= 100 mu m were defined as improved or worsened. Results Numerical changes in BCVA and EZ loss during follow-up were not statistically significant. However, the mean CMT at baseline, which was lower than that of healthy control eyes (P < 0.001), significantly increased during follow-up (P = 0.041). A certain proportion of eyes showed improvement in several parameters: funduscopic findings (both parafoveal retinal graying and foveal retinal pigment epithelium depigmentation) in 29% of eyes, CMT in 21% of eyes, and EZ loss in 43% of eyes. Conclusions The non-negligible proportion of eyes with improved parameters, marked especially by macular EZ loss, suggests that Japanese patients with MacTel-2 have milder clinical features than Caucasian patients reported in the literature.

Purpose To present a case of unilateral stellate nonhereditary idiopathic foveomacular retinoschisis in a patient with macular telangiectasia (MacTel) Type 2. Methods Single-patient case report. Results A 61-year-old female white patient was referred to our clinic with metamorphopsia and reduction of visual acuity over a period of 2 months on her right eye. Ocular findings in her right eye included reduced best-corrected visual acuity of 20/63 Snellen, foveomacular retinoschisis with extension to the lower middle periphery, central elevation of the neurosensory retina, and macular telangiectasia (MacTel) Type 2. Other causes of foveomacular retinoschisis, such as glaucoma, myopic degeneration, optic or scleral pit, X-linked juvenile retinoschisis, degenerative retinoschisis, and vitreomacular traction, were ruled out. The patient had no history of niacin or taxane medication, which may cause rather similar appearing cases of cystoid macular edema without leakage in fluorescein angiography. Because of the unilateral presentation, uneventful medical history, female sex, and the absence of known hereditary diseases or retinal pathologies in the patient's family history, hereditary predisposition appears to be highly unlikely. Conclusion To our knowledge, this is the first reported case of stellate nonhereditary idiopathic foveomacular retinoschisis in combination with MacTel Type 2. Whether or not MacTel Type 2 plays a role in the development of stellate nonhereditary idiopathic foveomacular retinoschisis or has an impact on its clinical course requires further investigation. Furthermore, we suggest a significant involvement of Henle fiber layer in the process of intraretinal expansion in optical coherence tomography, in accordance with the most recent published nomenclature.

Tyler A Berger,1 Matthew W Manry,1,2 Lucas B Lindsell,1,2 James M Osher,1,2 Daniel M Miller,1,2 Robert E Foster,1,2 Christopher D Riemann,1,2 Michael R Petersen,1,2 Robert A Sisk1– 3 1Department of Ophthalmology, University of Cincinnati, Cincinnati, OH, USA; 2Cincinnati Eye Institute, Cincinnati, OH, USA; 3Abrahamson Pediatric Eye Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USACorrespondence: Robert A SiskCincinnati Eye Institute, 1945 CEI Drive, Cincinnati, OH, 45242, USAFax +1 513-569-3904Email rsisk@cvphealth.comPurpose: To evaluate if off-label Age-Related Eye Disease Study 2 (AREDS2) supplementation prevents visual and anatomical deterioration in non-proliferative Idiopathic Macular Telangiectasia Type 2 (IMT2).Patients and Methods: This is a single-center retrospective, comparative study of 82 IMT2 eyes treated with AREDS2 from January 1st, 2013 to January 1st, 2018. The study analysis consisted of a non-comparative arm, which included all AREDS2 eyes, and a comparative arm (27 AREDS2 and 42 untreated eyes) that only included eyes with complete follow-up data. Eyes were evaluated at baseline, 12 and 24 months. Better/worse eye sub-analysis was performed in the comparative study arm. Primary outcomes were best corrected visual acuity (BCVA) and optical coherence tomography (OCT) anatomical characteristics including largest cavitation diameter, central macular thickness (CMT), and length of ellipsoid zone (EZ) loss at 24 months.Results: In the non-comparative arm, AREDS2 eyes showed stable BCVA (0.28 ± 0.18 logMAR at baseline vs 0.26 ± 0.19 logMAR at 24 months; p = 0.35) and OCT anatomical features after 24 months of supplementation. In the comparative arm, BCVA mean difference was greater for untreated eyes at 24 months (− 0.09 ± 0.15 vs 0.03 ± 0.11 logMAR; p = < 0.001). AREDS2 eyes had decreased cavitary diameter and EZ loss compared to untreated eyes at the study endpoint (p = 0.01 and p = 0.02, respectively). CMT remained stable for both cohorts throughout the study. For better/worse eye analysis, untreated eyes had worse BCVA at 24 months in both better and worse eyes (both p = 0.01). For anatomical outcomes, increases in both EZ loss (p = 0.04) and cavitary diameter (p = 0.001) among untreated eyes were only significant for eyes with worse baseline BCVA.Conclusion: Our results suggest that off-label AREDS2 supplementation in non-proliferative IMT2 may prevent anatomical and visual deterioration in a subset of eyes.Keywords: antioxidant, AREDS2, carotenoid supplementation, ellipsoid zone loss, lutein, idiopathic macular telangiectasia, macular telangiectasia, juxtafoveal macular telangiectasia, parafoveal telangiectasia, retinal vascular disease

Purpose To report a case of central retinal artery occlusion in a patient with macular telangiectasia Type 2 using multimodal imaging. Methods Observational case report. Results A 58-year-old woman who presented with acute painless unilateral vision loss was diagnosed with central retinal artery occlusion in her right eye with macular telangiectasia Type 2 findings in both eyes. Fundus examination revealed retinal whitening with unusual cherry-red spot plus small crystalline deposits in the temporal macula. Surprisingly, spectral domain optical coherence tomography of the contralateral eye showed characteristically intraretinal hyporreflective spaces, whereas optical coherence tomography angiography exhibited microvascular abnormalities in the deep capillary plexus. At follow-up, a pseudolamellar macular hole was noticed in the affected eye with no recovery of best-corrected visual acuity. Conclusion This case describes an onset of central artery occlusion in a patient with underlying macular telangiectasia Type 2 and suggests that it had a possible role in the acceleration of its natural course. The utility of multimodal imaging lies on better accuracy in diagnosis as well as prognosis, management, and monitoring of the disease.

We present a case of a woman with idiopathic macular telangiectasia type 1, characterized by the presence of a capillary macroanaeurysm associated with macular edema with a macular star pattern. Several differential diagnoses were proposed, in which the study with a multimodal image was key to clarify the diagnosis. In addition, the various treatments used and their effectiveness are discussed.

PURPOSE To longitudinally characterize structural/angiographic findings of retinal-choroidal anastomosis (RCA) in macular telangiectasia Type 2 (MacTel2). METHODS A combined retrospective/prospective review of patients with MacTel2 with >2 visits and >2 months follow-up, including examination, fundus photography, fluorescein angiography, spectral-domain optical coherence tomography, and projection-resolved optical coherence tomography angiography. RESULTS There were 24 eyes of 12 patients with a mean age of 61.1 (±7.3 [SD]) years and median follow-up of 447 (range 81-1,511) days. There were five eyes diagnosed with new RCA after not having any previously. Retinal-choroidal anastomosis was diagnosed in 8 (33%) eyes initially and eventually 13 (54%). In the eight eyes initially diagnosed with RCA, the number of RCAs and outer retinal hyperrflective lesions increased over the follow-up. The RCAs were found in clusters, generally temporal to the fovea. Retinal-choroidal anastomosis was uniformly associated with complete retinal subsidence, defined as the loss of outer nuclear layer substance such that the middle retinal layers sank down toward the retinal pigment epithelium (RPE), and was also present in each case. Each RCA colocalized with a region of hyperreflectivity, previously termed an outer retinal hyperreflective lesion. There was a lack of fluorescein leakage, lipid, hemorrhage, or any structural correlates with fluid exudation, and no signs of subretinal/subretinal pigment epithelium neovascularization. CONCLUSION De novo RCA development seems to be associated with retinal subsidence, and after RCAs form, they increase in number and topographic distribution over time. Initially after formation, there was no sign of exudation. These data suggest the descent of the deep capillary plexus occurs with RCA, devoid of subretinal/subretinal pigment epithelium neovascularization in MacTel2.

Introduction. The few published studies that exist on the surgical outcomes of full-thickness macular hole (FTMH) repair in Macular Telangiectasia (MacTel) Type 2 report poor rates of hole closure of around 30%. This study is the largest case series of patients with FTMH in MacTel Type 2 and describes an 80% hole closure rate. Purpose/Aim. To describe the outcomes of four patients who underwent surgery for FTMH associated with MacTel Type 2. Methods. A retrospective review of clinical, surgical, and imaging data of five eyes in four patients with MacTel Type 2 FTMH who underwent pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling and 30% SF6 or 15% C3F8 gas tamponade within 3-9 months of initial vision decline. Results. Visual acuity (VA) at the time of surgery ranged from 20/50 to 20/200. Successful hole closure was achieved in four out of five eyes, and final VA ranged from 20/20 to 20/40 at follow-up visits greater than 20 months postoperatively. The single eye that did not achieve hole closure had a final VA of 20/60. Conclusion. Our case series describes higher hole closure rates and better final VA than previously published reports for macular hole surgery in patients with MacTel Type 2 FTMH.

Purpose To report the visual acuity measures from the macular telangiectasia type 2 (MacTel) registry and to investigate and describe phenotypic findings in eyes with substantial vision loss resulting from MacTel. Design Cross-sectional multicenter study. Participants Participants in the MacTel Natural History Observation Registration Study. Methods Best-corrected visual acuity (BCVA) data, retinal imaging data, and clinical data were accessed from the MacTel Study databases in May 2019. Main Outcome Measures Frequency distribution of BCVA and its relationship to age; morphologic changes in eyes with very late disease stages, defined by a BCVA of 20/200 or worse; average retinal thickness of macular subfields on OCT; and dimensions of the area affected by MacTel (i.e., the MacTel area). Results Best-corrected visual acuity was 20/50 or worse in 37.3% and 20/200 or worse in 3.8% of 4449 eyes of 2248 patients; 18.4% and 0.7% of all patients showed bilateral BCVA of 20/50 or worse and 20/200 or worse, respectively. Asymmetry between right and left eyes was present (median BCVA, 71 letters vs. 74 letters), a finding supported by more advanced morphologic changes in right eyes. Participant age correlated with BCVA, but the effect size was small. If a neovascularization or macular hole were present, bilateral occurrence was frequent (33% or 17%, respectively), and BCVA was better than 20/200 (79% or 78%, respectively) or 20/50 or better (26% or 13%, respectively). Eyes with advanced disease (BCVA, ≤20/200) showed the following characteristics: (1) atrophy of the foveal photoreceptor layer with or without associated subretinal fibrosis; (2) an affected area, termed MacTel area, limited to a horizontal diameter not exceeding the distance between the temporal optic disc margin and foveal center, and the vertical diameter not exceeding approximately 0.8 times this distance (exceptions were eyes with large active or inactive neovascular membranes); (3) reduced retinal thickness measures within the MacTel area; and (4) less frequent retinal greying and more frequent hyperpigmentations compared with eyes that have better BCVA. Conclusions Severe vision loss is rare in MacTel and is related to photoreceptor atrophy in most people. Results indicate disease asymmetry with slightly worse vision and more advanced disease manifestation in right eyes. MacTel-related neurodegeneration does not spread beyond the limits of the MacTel area.

Purpose To evaluate changes in the visualization of microaneurysms (MAs) in cases of macular telangiectasia (Mac Tel) type 1 on optical coherence tomography angiography (OCTA) before and after treatment with direct photocoagulation and to evaluate their relationship with treatment efficacy. Methods The study included 12 eyes from 12 patients (8 men, 4 women; mean age 72.1 years) with Mac Tel type 1 accompanied by cystoid macular edema. OCTA for the evaluation of MAs was performed before and 15 min and 6, 12, and 24 weeks after photocoagulation. The best-corrected visual acuity (BCVA) and central retinal thickness (CRT) were also evaluated. Results A total of 73 MAs were detected within the areas of macular edema on OCTA, and 39 of these underwent photocoagulation. At 15 min after treatment, 17 MAs were no longer visible on OCTA. At 6 weeks, two MAs had reappeared, whereas five additional MAs were no longer visible. The CRT in eyes with resolved MA was significantly less than that in eyes with persistent MAs (p = 0.016). At 24 weeks, seven eyes had no visible MAs, and the BCVA was not significantly different from baseline. Conclusion OCTA can monitor changes in the visualization of MAs associated with Mac Tel type 1 after direct photocoagulation. Eyes in which MAs disappeared after treatment could recover from cystoid macular edema.

We analysed the imaging findings of macular telangiectasia (MacTel) type 2 in Korea using spectral domain optical coherence tomography (SD-OCT) and investigated their relationship with visual acuity and clinical stages. A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and included 129 patients. We analysed all the SD-OCT images encompassing the macular area. Hyporeflective cavities (77.7%) were the most frequently detected abnormalities in SD-OCT. Disruption of the external limiting membrane, ellipsoid zone, and interdigitation zone were found in 67 (40.4%), 87 (52.4%), and 94 eyes (56.6%), respectively. Four eyes (2.4%) had lamellar macular hole, and five eyes (3.0%) full-thickness macular hole. Neovascularisation, either subretinal or intraretinal, was found in 14 eyes (8.4%). Eyes with outer retinal hyperreflective band disruption had lower visual acuity than those without them. The presented characteristic clinical features of OCT in MacTel type 2 can not only aid in differentiating this disease from others but are also helpful for better judgement of the disease stage in daily clinical practice. Inner retinal hyporeflective cavities without outer retinal abnormalities on SD-OCT, although classified as severity scale 3, could be considered a relatively early stage in the disease process in terms of vision.

Purpose: To report the characteristics of non-proliferative fellow eyes in patients with unilateral proliferative macular telangiectasia type 2 (MacTel). Methods: This was a multicenter, retrospective study and included fellow eyes of eyes with subretinal neovascular membrane (SRNVM). Multimodal imaging including fundus photography, optical coherence tomography (OCT), and OCT angiography (OCTA) was performed. OCT and OCTA measurements included central macular thickness (CMT), subfoveal choroidal thickness (SFCT), foveal avascular zone (FAZ) area and qualitative parameters such as distortion or enlargement of FAZ, increased inter-vascular spacing, dilated, tortuous vessel at margin of FAZ, crowding of vessel, and loss of choriocapillaris (CC). Results: Thirteen fellow eyes of 13 patients with mean age of 63.6 ± 12.0 years were included. Fellow eyes had various stages of the diseases – stage 2 (four eyes), 3 (six eyes), and 4 (three eyes). OCTA showed increased intervascular spacing (8; 61.5%), dilated (11; 84.6%), and elongated vessels (8; 61.5%) at the margin of the FAZ, crowding of vessels (6; 46.2%), capillary non-perfusion areas outside the FAZ (4; 30.8%), and areas of CC loss (4; 30.8%). Mean follow-up was 9.1 ± 12.9 months. Out of six eyes with follow-up of ⩾12 months, two developed SRNVM at 18 and 33 months with OCTA showing crowding and entanglement of dilated retinal vessels in deep capillary plexus at baseline which corresponded to the area affected by SRNVM. Conclusion: Fellow eyes of proliferative MacTel showed established disease stages on OCTA with progressive capillary crowding and entanglement of vessels likely corresponding to the future site of SRNVM.

Background Optical coherence tomography angiography (OCT-A) has emerged as a novel, fast, safe and non-invasive imaging technique of analyzing the retinal and choroidal microvasculature in vivo. OCT-A captures multiple sequential B-scans performed repeatedly over a specific retinal area at high speed, thus enabling the composition of a vascular map with areas of contrast change (high flow zones) and areas of steady contrast (slow or no flow zones). It therefore provides unique insight into the exact retinal or choroidal layer and location at which abnormal blood flow develops. OCTA has evolved into a useful tool for understanding a number of retinal pathologies such as diabetic retinopathy, age-related macular degeneration, central serous chorioretinopathy, vascular occlusions, macular telangiectasia and choroidal neovascular membranes of other causes. OCT-A technology is also increasingly being used in the evaluation of optic disc perfusion and has been suggested as a valuable tool in the early detection of glaucomatous damage and monitoring progression. Objective To review the existing literature on the applications of optical coherence tomography angiography in neurodegenerative diseases. Summary A meticulous literature was performed until the present day. Google Scholar, PubMed, Mendeley search engines were used for this purpose. We used 123 published manuscripts as our references. OCT-A has been utilized so far to describe abnormalities in multiple sclerosis (MS), Alzheimer's disease, arteritic and non-arteritic optic neuropathy (AION and NAION), Leber's hereditary optic neuropathy (LHON) papilloedema, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis (ALS), Wolfram syndrome, migraines, lesions of the visual pathway and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). It appears that OCT-A findings correlate quite well with the severity of the aforementioned diseases. However, OCT-A has its own limitations, namely its lack of wide-field view of the peripheral retina and the inaccurate interpretation due to motion artifacts in uncooperative groups of patients (e.g. children). Larger prospective longitudinal studies will need to be conducted in order to eliminate the aforementioned limitations.

Purpose: This work reports long-term outcomes in macular telangiectasia type 2 (MacTel) with subretinal neovascularization (SRNV). Methods: A retrospective, single-center review of medical records was performed on all patients with a diagnosis of MacTel presenting between May 2004 and October 2019. Medical and ocular history, best-corrected visual acuity (BCVA) at baseline and final visit, optical coherence tomography data, and treatment history of SRNV secondary to MacTel were recorded. Results: A total of 471 eyes were diagnosed with MacTel. SRNV was present in 44 eyes (9.3%), of which 38 eyes met inclusion criteria for SRNV. Average follow-up duration in the SRNV group was 78.4 months. All SRNV patients underwent antivascular endothelial growth factor (anti-VEGF) therapy. There was no significant change from mean baseline (0.59 ± 0.45) to final (0.70 ± 0.49) BCVA in the SRNV group as a whole ( P = .13). Subgroup analysis revealed 17 of 38 eyes had SRNV at diagnosis and received immediate anti-VEGF treatment. In this subgroup mean pretreatment BCVA was 0.89 ± 0.43 and the mean final BCVA was 0.87 ± 0.61 ( P = .84). The remainder (21 of 38 eyes) developed SRNV during follow-up. In this subgroup, final BCVA after initiation of treatment was 0.56 ± 0.32, an improvement in BCVA from SRNV onset ( P = .04) and a decrease from pre-SRNV onset baseline BCVA ( P = .008). Conclusions: Visual acuity is maintained, not improved, in long-term follow-up of MacTel with SRNV treated with anti-VEGF. Patients presenting with SRNV have a worse prognosis than those who develop SRNV during follow-up.

Purpose To compare visual acuity (VA) and OCT outcomes in patients with idiopathic macular telangiectasia (IMT) type 2 who underwent pars plana vitrectomy (PPV) surgery for full-thickness macular holes (FTMHs) versus those who elected to be medically managed (MM) without surgery. Design Comparative retrospective case series. Participants Patients with IMT type 2 and FTMH. Methods We reviewed records within an 11-year period and collected data on VA, OCT changes, development of choroidal neovascularization, and length of follow-up. The VA measurements were standardized from Snellen to logarithm of the minimum angle of resolution units for statistical analysis. Two-sample t tests were used to analyze VA data. OCT changes were assessed by a single masked retinal specialist. Results There were 12 eyes in the PPV group and 26 eyes in the MM group. There was no statistically significant VA improvement in either group between initial VA recording and last follow-up. The PPV group had no significant change in VA between the preoperative visit and the visits at 3 or 12 months. OCT scans improved by 1 step in 10 patients in the PPV group. None of the patients in the MM group had OCT improvement. Choroidal neovascularization developed in 1 eye in the PPV group and 5 eyes in the MM group. Conclusions There was no significant change in VA in patients who opted to have PPV to treat their IMT type 2 and FTMH compared with those who did not undergo surgery. OCT scans improved by qualitative judgment in patients who underwent surgery compared with those who opted for medical management.

Background/aimsTo quantify retinal cavitation size over time in macular telangiectasia type 2 (MacTel) and to correlate changes with visual acuity and area of ellipsoid zone loss.MethodsOptical coherence tomography (OCT) macula volume scans from sham eyes included in a prospective, phase II clinical trial of human ciliary neutrophic factor for MacTel at baseline, 1 year and 2 years of follow-up were analysed. Cavitations were segmented by two independent readers. Total cavitation volume was compared with area of ellipsoid zone loss and best-corrected visual acuity (BCVA).ResultsFifty-one eyes from 51 unique patients (mean age 62 years, range 45–79 years) were included. Intraclass correlation between readers for cavitation volume was excellent (>0.99). Average cavitation volume was 0.0109 mm3, 0.0113 mm3and 0.0124 mm3at baseline, 1 year and 2 years, respectively. The average rate of cavitation volume change was +0.0039 mm3/year. 10 eyes (20%) had a significant change in cavitation volume during the study (3 decreased, 7 increased). Eyes with increased cavitation volume had worse BCVA compared with eyes with no change/decreased cavitation volume (71.5 vs 76.1 ETDRS letters, respectively). Cavitation volume was negatively correlated to BCVA (r=−0.37) but not to area of ellipsoid zone loss. Cavitation volume was negatively predictive of BCVA in both univariate and multivariate mixed-effects modelling with ellipsoid zone loss.ConclusionsRetinal cavitations and their rate of change in MacTel can be reliably quantified using OCT. Cavitations are negatively correlated with visual acuity and may be a useful OCT-based biomarker for disease progression and visual function in MacTel.

Supplemental Digital Content is Available in the Text. This study confirms that fundus autofluorescence lifetimes display characteristic patterns in patients with MacTel Type 2 disease and provide information about macular pigment reduction and possibly photoreceptor loss. Fluorescence lifetime imaging ophthalmoscopy offers a novel diagnostic tool in the investigation of eyes with MacTel and has the potential of becoming a noninvasive screening modality., Purpose: Type 2 idiopathic macular telangiectasia (MacTel) is a rare bilateral neurodegenerative disease characterized by alterations in the macular capillary network leading to central vision loss. The purpose of this study was to quantify disease-specific retinal fluorescence lifetime patterns in patients with MacTel using fluorescence lifetime imaging ophthalmoscopy. Participants: Both eyes of 14 patients (mean age ± SEM, 67.8 ± 6.4 years) with a clinical diagnosis of MacTel Type 2 and 14 healthy age-matched controls (age 69.8 ± 6.4 years) were included in this study. Methods: All participants were imaged with a fluorescence lifetime imaging ophthalmoscope (Heidelberg Engineering, Germany). Mean retinal fluorescence lifetimes (Ƭm) were obtained in the short spectral channels (498–560 nm) and long spectral channels (560–720 nm). Clinical features, fundus images, fundus autofluorescence intensity images, spectral domain optical coherence tomography, and corresponding macular pigment optical density measurements using a modified confocal scanning laser ophthalmoscope (mpHRA) were further analyzed. Patients were classified into five phenotypic subgroups using the Gass and Blodi classification. Results: Mean fluorescence lifetimes were significantly prolonged temporal to the fovea in patients with MacTel compared with healthy controls (mean ± SEM: short spectral channels 543 ± 61 ps vs. 304 ± 9 ps; P < 0.0001; long spectral channels: 447 ± 26 ps vs. 348 ± 11 ps; P < 0.0001), and appeared as a crescent or ring-shaped pattern. Prolonged lifetime patterns correlated with decreased macular pigment density on macular pigment optical density measurements. Follow-up examinations were performed in four MacTel patients, which revealed an increase of short spectral channel Ƭm of 22% over 2.1 years in the temporal fovea. Conclusion: This study confirms that fundus autofluorescence lifetimes display characteristic patterns in patients with MacTel Type 2 disease and provide information about macular pigment and possibly photoreceptor loss. Fluorescence lifetime prolongation correlates with disease severity and may therefore be a useful addition to other imaging modalities for assessing disease progression in MacTel Type 2.

PURPOSE To assess the impact of neurodegenerative morphologic alterations due to macular telangiectasia type 2 (MacTel) on microperimetry (MP) and multifocal electroretinography (mfERG). METHODS Thirty-five eyes of 18 patients with MacTel were examined using spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), mfERG and MP. Software was used to match SD-OCT B-scans with the corresponding retinal sensitivity map and multifocal electroretinograms (mfERGs), thus enabling direct structure/function correlation. RESULTS Loss of the ellipsoid zone (EZ) had the strongest negative association with retinal sensitivity (16.77 dB versus 4.58 dB, adj. p

Significance Neuroretinitis classically presents with sudden vision loss, disc edema, and macular star formation. However, the classical triad may not always be seen, especially in the case of a pre-existing macular disorder. A thorough clinical examination may still clinch the appropriate diagnosis and prevent unwarranted treatment. Purpose This study aimed to report a case of neuroretinitis in an adult woman with pre-existing type 2 macular telangiectasia where the classical pattern of exudation could not be seen. Case report A 48-year-old woman, type 2 diabetic and hypertensive, presented with sudden painless blurring of vision in the right eye for the past 1 week. Macula in both eyes had loss of transparency and intraretinal crystalline deposits and pigments. The right eye had hyperemic edematous disc with peripapillary retinal hemorrhages and hard exudates. Color vision was grossly abnormal, and a centrocecal scotoma was noted on visual field analysis in the right eye. After a normal systemic evaluation, a diagnosis of right eye neuroretinitis and coexistent bilateral type 2 macular telangiectasia (nonproliferative type) was made. Unilateral painless visual loss, severe dyschromatopsia, and peripapillary hard exudates pointed to the correct diagnosis of neuroretinitis. Observation was advised, and visual acuity improved spontaneously. The disc edema resolved with consequent optic atrophy. Conclusions Absence of the classical "macular star" appearance does not refute the diagnosis of neuroretinitis when pre-existing maculopathy is present.

ObjectiveIdiopathic macular telangiectasia (MacTel) is considered primarily a vascular disease affecting juxtafoveal retinal capillaries. However, recent evidence suggests that neuronal changes may occur early in disease development. We used high-resolution adaptive optics retinal imaging to elucidate the foveal cone photoreceptor changes at a cellular level in patients with MacTel.Methods and analysisWe used adaptive optics scanning light ophthalmoscopy (AOSLO) to evaluate the foveal cone photoreceptors in the less-affected eye of patients with asymmetric MacTel. AOSLO images of cone photoreceptors were obtained in a 4°×4° area centred on the foveola. Individual cone positions were identified within a 2°×2° area centred on the fovea, using semiautomatic cone marking software with manual correction, permitting calculation of a map of cone density.ResultsIn all participants, one eye was affected with MacTel, the fellow eye was clinically normal or near normal, with visual acuity of 20/25 or better and subtle angiographic leakage. The foveal cone mosaics were continuous with tight packing and cones exhibited normal reflectivity. However, cone density was significantly lower for all participants (mean=80 733 cones/mm2) within 0.5° than the cone density previously reported for normal eyes.ConclusionsFoveal cone density is lower than normal in the clinically less-affected eyes of patients with asymmetric MacTel. This suggests that cone photoreceptor loss may precede classic obvious vascular changes in idiopathic MacTel.

Purpose Fluorescence lifetime imaging ophthalmoscopy (FLIO) is a non-invasive imaging modality to investigate the human retina. This study compares FLIO lifetimes in different degenerative retinal diseases. Methods Included were eyes with retinal pigment epithelium (RPE) and/or photoreceptor atrophy due to Stargardt disease (n = 66), pattern dystrophy (n = 18), macular telangiectasia type 2 (n = 49), retinitis pigmentosa (n = 28), choroideremia (n = 26), and geographic atrophy (n = 32) in age-related macular degeneration, as well as 37 eyes of 37 age-matched healthy controls. Subjects received Heidelberg Engineering FLIO, autofluorescence intensity, and optical coherence tomography imaging. Amplitude-weighted mean FLIO lifetimes (τm) were calculated and analyzed. Results Retinal FLIO lifetimes show significant differences depending on the disease. Atrophic areas in geographic atrophy and choroideremia showed longest mean FLIO lifetimes. τm values within areas of RPE and outer nuclear layer atrophy were significantly longer than within areas with preserved outer nuclear layer (P < 0.001) or non-atrophic areas (P < 0.001). Conclusions FLIO is able to contribute additional information regarding differences in chronic degenerative retinal diseases. Although it cannot replace conventional autofluorescence imaging, FLIO adds to the knowledge in these diseases and may help with the correct differentiation between them. This may lead to a more in-depth understanding of the pathomechanisms related to atrophy and types of progression. Translational Relevance Differences between atrophic retinal diseases highlighted by FLIO may indicate separate pathomechanisms leading to atrophy and disease progression.

Purpose To describe findings of multimodal imaging in non-proliferative and proliferative stages of MacTel 2 in a pediatric patient, and results of aflibercept use for treating neovascularization secondary to MacTel 2. Methods Retrospective case report. Results An 11-year-old girl with no history of systemic disease. BCVA was 20/200 and 20/40 in the right and left eyes, respectively. FFA, SS-OCT and SS-OCTA revealed proliferative and non-proliferative stages of MacTel 2 in the right and left eyes, respectively. Intravitreal aflibercept (2mg/0.05mL) was started (PRN) in the right eye. The patient received 5 injections that led to involution of macular neovascularization and improvement of BCVA by 5 lines. BCVA in the left eye remained stable. Conclusion MacTel 2 can develop in an earlier age than previously reported. SS-OCTA is an effective alternative to conventional imaging in diagnosis and follow-up especially in pediatric patients. Intravitreal aflibercept is effective in treating proliferative MacTel 2.

Purpose We aimed to describe the vascular changes in eyes associated with tamoxifen retinopathy using OCT angiography (OCTA) and to compare these changes with abnormalities in macular telangiectasia type 2 (MacTel 2) previously reported in the literature. Design Retrospective, observational study. Participants Seventeen eyes with tamoxifen retinopathy and 17 eyes of age-matched healthy control participants. Methods The medical records of patients who visited the ophthalmology department with a history of taking tamoxifen were reviewed. Tamoxifen retinopathy was diagnosed based on typical spectral-domain (SD) OCT findings, such as intraretinal cavitation, photoreceptor disruption, or both. Multimodal imaging, particularly focused on OCTA, was analyzed. To compare vessel density in OCTA, age-matched normal control participants also were enrolled. Main Outcome Measures Descriptive appraisal of the vascular abnormalities and objective quantification of vessel density associated with tamoxifen retinopathy. Results Among 292 patients who were screened, 26 were diagnosed with tamoxifen retinopathy. Of these, 17 eyes of 10 patients who were evaluated using OCTA were included. All patients were women, with a median patient age of 65.0 years. They were treated with tamoxifen as adjuvant endocrine therapy for breast cancer. All eyes showed intraretinal cavitation, and 8 eyes showed focal photoreceptor disruption as well, on OCT. On OCTA imaging, 14 eyes (82.4%) showed saccular capillary telangiectasia at the deep capillary plexus and 6 eyes (35.3%) showed right-angled vessels. Foveal vessel density of the superficial plexus was significantly lower in eyes with tamoxifen retinopathy than in control participants (P = 0.003). Crystalline deposits on fundus photographs (12 eyes [70.6%]) and increased autofluorescence on fundus autofluorescence (16 eyes [94.1%]) also were noted as characteristic findings of tamoxifen retinopathy. Conclusions In addition to morphologic changes of tamoxifen retinopathy in SD OCT, its vascular changes on OCTA, such as telangiectatic vascular change at the deep capillary plexus and right-angled vessels, are similar to those observed in the early stages of MacTel 2.

Purpose To report a novel finding of epiretinal neovascularization (ERN) in cases of macular telangiectasia (MacTel) type 2 and describe its clinical and multimodal imaging features. Design Retrospective chart review. Participants Patients attending the retina clinic from January 2017 through April 2018. Those diagnosed with MacTel by clinical and imaging findings were included. Methods Best-corrected visual acuity in logarithm of minimum angle of resolution units, anterior segment examination, slit-lamp biomicroscopy, fundus photography, spectral-domain OCT, autofluorescence imaging, fundus fluorescein angiography, and OCT angiography (OCTA) were performed in all MacTel patients. Length of ellipsoid zone disruption, external limiting membrane disruption, area of the ERN in square millimeters, and central macular thickness in micrometers were noted. Main Outcome Measures The finding of an epiretinal membrane on spectral-domain OCT and a corresponding well-defined vascular complex on the vitreoretinal interface on OCTA in MacTel patients was considered to be evidence of epiretinal neovascular membrane (ERNM). Results Sixty-eight MacTel eyes underwent multimodal imaging, of which 7 eyes of 4 patients were identified as having ERN or ERNM. The ratio of men to women was 1:1 (n = 4); 1 patient had unilateral disease and 3 patients had bilateral disease. Mean age was 56.75 ± 10.71 years. Mean best-corrected visual acuity was 0.74 ± 0.39 logarithm of the minimum angle of resolution. Retinal pigment and dipping venules were present in 100% of eyes. Diffuse thinning and collapse sign were seen in all eyes. OCT angiography revealed a vascular membrane owing to the ERNM at the vitreoretinal interface. Mean area of the ERNM was 0.44 ± 0.248 mm2. This vascular membrane showed a demonstrable communication with the intraretinal abnormal vascular plexus in 6 of 7 eyes. None of the eyes showed a choroidal neovascular membrane. Conclusions Epiretinal neovascularization is a novel finding in MacTel type 2. These neovascular membranes are visualized best using OCT and OCTA. They are associated with retinal pigment and diffuse retinal thinning. Histopathologic studies are needed to characterize these membranes further.

To investigate the different appearances of vascular changes in macular telangiectasia type 2 (MacTel) and to describe their possible progression, the vascular patterns in different retinal layers were analyzed with optical coherence tomography angiography (OCT-A) and the findings were correlated with a spectral-domain OCT (SD-OCT) disease severity scale based on the extent of ellipsoid zone (EZ) loss. Participants from the MacTel Study Group in Muenster and a healthy cohort were investigated with OCT-A using RTVue XR Avanti. After segmentation of the superficial capillary network, the deep capillary network, and the outer retina (OR), flow density was analyzed using Optovue software. Then, the images were exported using the software Fiji (National Institute of Mental Health, Bethesda, MD, USA) and analyzed with the automated MATLAB program (Mathworks, Version R2014b). Four parameters (total vessel length, number of vessel branches, number of vessel segments, and fractal dimension) were examined on the vascular skeletons (temporal, foveal, nasal, and total fields of the ETDRS grid). In addition, linear and area measurements of EZ loss were performed on SD-OCT volume scans. Progression characteristics and correlation between linear and area measurements were analyzed using linear mixed effects models. Twenty eyes of healthy probands (20 OCT-A and 20 SD-OCT scans) and 122 eyes of 61 MacTel patients were included. In order to classify the severity of the disease, MacTel eyes were assigned to a SD-OCT “disease severity scale” (grade 1 = no EZ loss; grade 2 = EZ loss temporal to the fovea; grade 3 = EZ loss including the fovea and the region nasal to the fovea). Flow density and total vessel length showed only limited differences between healthy eyes and different grades of MacTel, but particularly the numbers of branches and vessel segments, as well as the fractal dimension values, demonstrated significant and progressive reduction in the superficial and deep capillary networks of the temporal, nasal, and total ETDRS fields. Moreover, the outer retina showed a progressive presence of hyperreflective material in SD-OCT grades 2 and 3 eyes with associated vascular patterns in the OR on OCT-A. In SD-OCT, the severity of MacTel is characterized by progressive EZ loss, which may be used as a simple clinical “disease severity scale”. In addition, OCT-A enables visualization and quantification of vascular patterns with mathematical methods. The morphological progression of the disease correlated significantly with progressive vascular changes, especially in respect of the numbers of branches and vessel segments as well as fractal dimension. This suggests that the severity of neurodegenerative and neurovascular changes develops in parallel and that the analysis and quantification of the vascular changes in the superficial and deep capillary networks may become an additional parameter for future treatment trials. Moreover, the significant association between hyperreflective material progressively visible on SD-OCT in the OR, which most often contains vessels in OCT-A, and advancing SD-OCT severity grades, as well as vascular changes in OCT-A, supports the concept of retinal neovascularization in the OR in patients with advanced MacTel.

PurposeTo evaluate the role of right-angled vessels (RAVs) during disease progression in macular telangiectasia type 2 (MacTel).MethodsIn this study, 100 eyes of 52 patients and 52 eyes of 26 age-related controls were examined using fundus photography, spectral-domain optical coherence tomography (SD-OCT), OCT angiography (OCT-A) and fundus fluorescein angiography (FFA). Two masked readers graded fundus photographs of patients’ eyes into five disease stages according to Gass and Blodi, and evaluated all eyes for the presence of RAVs. If RAVs were present, their course and origin (arterial vs venous) was evaluated with OCT-A and FFA, respectively. Additionally, we looked for morphological correlates of these vessels on SD-OCT scans. Neovascular eyes were analysed for the presence of RAVs and for morphological changes on formation of neovascularisations (NVs).ResultsIn OCT-A, RAVs were already detectable in eyes with early stages (1 to 2), could be tracked from superficial to outer retinal layers and were shown to form anastomoses in the outer retina with disease progression. These vessels were of both arterial and venous origin as shown by early phase FFA. Dilated capillaries and RAVs in OCT-A corresponded to hyper-reflective alterations of the outer retina on SD-OCT scans. In 19/19 eyes, NVs were associated with the presence of RAVs, and RAVs were shown to directly connect to neovascular complexes and to undergo morphological changes upon NV formation.ConclusionsThe results emphasise the role of RAVs during disease progression from an early stage on and demonstrate their involvement in the development of secondary NVs in MacTel.

Idiopathic macular telangiectasia type 2 (IMT2) is a relatively uncommon clinical condition with an estimated prevalence of 1% within the general population. This condition can be challenging to precisely identify in early stages but advancements in clinical imaging to include fluorescein angiography and optical coherence tomography angiography (OCTA) can allow for timely diagnosis and prompt intervention that may leads to improved long-term clinical outcomes. Emerging literature has recognized the role of macular pigment (MP) in IMT2 in terms of Henle fiber layer deposition mechanisms and potential mitigation of inflammatory and oxidative stress. Primary care optometrists are in a unique position to facilitate early detection and manage through close evaluation and individualized lutein and zeaxanthin supplementation. Keywords:Idiopathic macular telangiectasia type 2; Macular pigment; Spectral domain optical coherence tomography angiography

To evaluate the retinal and choroidal vascular changes through optical coherence tomography angiography (OCTA) in patients with macular telangiectasia type 2 (MacTel 2). Our study included 20 patients (40 eyes) with MacTel 2, and age-matched and sex-matched 18 subjects (36 eyes) in the control group. Fundus color photographs, fundus autofluorescence, fundus fluorescein angiography, spectral-domain optical coherence tomography and OCTA were performed. Foveal vascular density and parafoveal vascular density (PFVD), and foveal retinal thickness and parafoveal retinal thickness, choroidal thickness (CT) and retinal ganglion cell–inner plexiform layer (GCIPL) were compared between MacTel 2 patients and normal age-matched controls. The retinal whole vascular density and PFVD of the deep plexus were significantly lower in patients with MacTel 2 than that of the control group (56.93% vs. 58.54%, p = 0.003; and 60.38% vs. 61.66%, p = 0.045). The foveal avascular zone (FAZ) of the deep plexus was significantly enlarged in patients with MacTel 2 than that of the control group (0.44 vs. 0.36, p = 0.009). There was a positive and statistically significant correlation between the FAZ of the superficial and deep plexus and CT in patients with MacTel 2. There was a positive and statistically significant correlation between retinal whole, parafoveal temporal quadrant vascular density of the superficial and deep plexus and GCIPL thickness in patients with MacTel 2. Our study demonstrated that important retinal vascular density and FAZ changes in MacTel 2 occur in the deep capillary plexus of the retina.

PurposeTo investigate the choroidal thickness (CT) and choroidal vascular densities (CVD) of patients with macular telangiectasia type 2 (MacTel2) and their association with other multimodal imaging features, using swept-source optical coherence tomography (SS-OCT).MethodsProspective, cross-sectional study. Consecutive patients with MacTel2 along with controls without any macular disease were included. Fundus photography, confocal blue reflectance, near-infrared reflectance, autofluorescence, fluorescein angiography, spectral domain OCT and SS-OCT were performed. Images were independently analysed by two graders, and CVD was calculated from binarised en face SS-OCT images. CT was obtained from the SS-OCT platform via built-in automated segmentation. Multilevel mixed-effects models were used for statistical analysis.ResultsThirty-nine eyes of 20 patients with MacTel2 and 29 eyes of 15 control patients were included. Average CT and perifoveal temporal CT did not differ significantly between eyes with MacTel2 and control eyes (p≥0.350), when accounting for confounding factors. Overall and temporal CVD also did not significantly differ between the two groups (p≥0.490).ConclusionCT and CVD did not significantly differ between MacTel2 and control eyes in this study using SS-OCT. Even though MacTel2 may include abnormalities involving the choroid, these are likely minor in comparison to the predominant retinal changes.

To study clinical and imaging features of various stages of macular telangiectasia (MacTel type 2). In this retrospective study, cases of MacTel type 2 with fluorescein angiography (FA), optical coherence tomography (OCT) and OCT-angiography (OCTA) imaging were included. Based on angiographic perifoveal fluorescence, two groups were formed: group 1: diffuse hyperfluoroscence and group 2: diffuse + focal hyperfluoroscence. Later, based on OCT features, group 2 was subdivided into group 2A: without SRNVM and group 2B: with SRNVM. Clinical, FA, OCT and OCTA features were analysed. Eyes showing conversion to the proliferative stage at final visit were noted. Ninety-four eyes of 48 patients were included. Group 1 (n = 28) showed diffuse perifoveal hyperfluoroscence, hyperreflective middle retinal layers, absent SRNVM (p = 0.006) on OCT and dilated perifoveal capillaries in deep capillary plexus (DCP) on OCTA. Group 2A (n = 40) showed diffuse + focal perifoveal hyperfluoroscence, hyperreflective middle retinal layers (p = 0.001), hyporeflective outer retina cavities (p = 0.021), absent SRNVM with dilated and bunching perifoveal capillaries (p = 0.004) in DCP. Group 2B (n = 26) showed late diffuse + focal perifoveal hyperfluoroscence, foveal contour irregularity (p = 0.002), retinal pigment clumps (p = 0.015) and SRNVM on OCT with bunching of capillaries in DCP and vessels in outer retina (p = 0.002). Five eyes showed conversion to group 2B at final visit. There exists a distinct disease stage called “preproliferative” MacTel type 2 showing clinical features of non-proliferative disease, diffuse + focal perifoveal hyperfluoroscence on FA, absent SRNVM on OCT and bunching perifoveal capillaries in DCP on OCTA. Its identification is important for suspecting proliferative disease, planning management and follow-up visit accordingly.

Tyler A Berger,1 Matthew W Manry,1,2 Lucas B Lindsell,1,2 James M Osher,1,2 Daniel M Miller,1,2 Robert E Foster,1,2 Christopher D Riemann,1,2 Michael R Petersen,1,2 Robert A Sisk1– 3 1Department of Ophthalmology, University of Cincinnati, Cincinnati, OH, USA; 2Cincinnati Eye Institute, Cincinnati, OH, USA; 3Abrahamson Pediatric Eye Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USACorrespondence: Robert A SiskCincinnati Eye Institute, 1945 CEI Drive, Cincinnati, OH, 45242, USAFax +1 513-569-3904Email rsisk@cvphealth.comPurpose: To evaluate if off-label Age-Related Eye Disease Study 2 (AREDS2) supplementation prevents visual and anatomical deterioration in non-proliferative Idiopathic Macular Telangiectasia Type 2 (IMT2).Patients and Methods: This is a single-center retrospective, comparative study of 82 IMT2 eyes treated with AREDS2 from January 1st, 2013 to January 1st, 2018. The study analysis consisted of a non-comparative arm, which included all AREDS2 eyes, and a comparative arm (27 AREDS2 and 42 untreated eyes) that only included eyes with complete follow-up data. Eyes were evaluated at baseline, 12 and 24 months. Better/worse eye sub-analysis was performed in the comparative study arm. Primary outcomes were best corrected visual acuity (BCVA) and optical coherence tomography (OCT) anatomical characteristics including largest cavitation diameter, central macular thickness (CMT), and length of ellipsoid zone (EZ) loss at 24 months.Results: In the non-comparative arm, AREDS2 eyes showed stable BCVA (0.28 ± 0.18 logMAR at baseline vs 0.26 ± 0.19 logMAR at 24 months; p = 0.35) and OCT anatomical features after 24 months of supplementation. In the comparative arm, BCVA mean difference was greater for untreated eyes at 24 months (− 0.09 ± 0.15 vs 0.03 ± 0.11 logMAR; p = < 0.001). AREDS2 eyes had decreased cavitary diameter and EZ loss compared to untreated eyes at the study endpoint (p = 0.01 and p = 0.02, respectively). CMT remained stable for both cohorts throughout the study. For better/worse eye analysis, untreated eyes had worse BCVA at 24 months in both better and worse eyes (both p = 0.01). For anatomical outcomes, increases in both EZ loss (p = 0.04) and cavitary diameter (p = 0.001) among untreated eyes were only significant for eyes with worse baseline BCVA.Conclusion: Our results suggest that off-label AREDS2 supplementation in non-proliferative IMT2 may prevent anatomical and visual deterioration in a subset of eyes.Keywords: antioxidant, AREDS2, carotenoid supplementation, ellipsoid zone loss, lutein, idiopathic macular telangiectasia, macular telangiectasia, juxtafoveal macular telangiectasia, parafoveal telangiectasia, retinal vascular disease

Purpose: To investigate the demographic and multimodal imaging features of macular telangiectasia (MacTel) type 2 in Korea and their relationship with visual acuity and the clinical stage. Methods: A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and the study included 84 patients. Demographic data and imaging data of fundus photography, fundus autofluorescence (FAF), confocal blue-light reflectance (CBR), fluorescein angiography (FAG), and optical coherence tomography (OCT) were collected. Results: The Korean patients with MacTel type 2 were predominantly female (75%), and the mean logMAR visual acuity was 0.282 ± 0.280 at initial presentation. Most commonly presented signs were the loss of retinal transparency in fundus photographs (68.3%); increased autofluorescence in FAF (83.6%); increased blue reflectance involving the centre in CBR (68.0%); telangiectatic vessels in FAG (88.2%); and hyporeflective cavities in OCT (77.7%). The eyes diagnosed in the first half of the study period (2009–2014) showed a tendency to be diagnosed at more advanced severe stages than those diagnosed in the second half of the study period (2015–2019), using new severity scales based on FAG, FAF and OCT findings. Conclusion: The clinical features of MacTel type 2 in Korean patients assessed by newer imaging modalities suggest that Korean patients and the Caucasian-dominant population show similar presentations. This study showed that MacTel type 2 can be diagnosed in the earlier phase of the disease by using new imaging modalities and through better understanding of the disease.

BackgroundMacular telangiectasia type 2 (MacTel) is a neurodegenerative disease resulting in photoreceptor loss. Optical coherence tomography (OCT) reveals outer retina-associated hyperreflectivity (ORaH) as part of this process. The purpose of this study was to describe the incidence and phenotypical variation of ORaH.MethodsDifferent parameters of ORaH were analysed: OCT characteristics (Spectralis SD-OCT), correlation with vascular changes (OCT angiography; OCTA 3×3 mm Optovue) and correlation with hyperpigmentation (autofluorescence/fundus images). ORaH was also evaluated regarding the grade of severity of photoreceptor loss (Disease Severity Scale).ResultsOf 220 eyes with MacTel type 2, 106 demonstrated ORaH. On OCT, the size, the extension into the inner retina and the contact with retinal pigment epithelium (RPE) of the ORaH were variable. On OCTA neovascularisation (NV) in the outer retina (OR) was present at the location of the ORaH in 97.6%. Increasing size of NV correlated with progressive photoreceptor loss. In 86.6% with NV, the flow signals were visible between the OR and the choriocapillaris. In 85.7%, the ORaH was associated with hyperpigmentation on autofluorescence and fundus colour images.ConclusionsThe presence of ORaH is associated with increasing photoreceptor loss and disease severity. In these more advanced cases of the present study, a variable presentation of ORaH in respect to size and form was seen, but in most cases, ORaH was in contact to the RPE. Additionally, ORaH was associated with hyperpigmentation and OR NV on OCTA. These results are consistent with the concept of ORaH representing fibrovascular OR-NV with RPE proliferation after contact with the RPE.

Purpose To investigate the relationship between disruption in different photoreceptor layers and deep capillary plexus (DCP) telangiectasias in eyes with macular telangiectasia type 2 (MacTel). Methods 35 eyes (21 patients) with MacTel imaged with optical coherence tomography angiography (OCTA) were included. Circumscribed areas of DCP telangiectasia were traced from OCTA slabs and the corresponding spectral-domain OCT (SD-OCT) slabs were used to visualize the photoreceptor layer interdigitation zone (IZ) and ellipsoid zone (EZ). IZ attenuation, IZ loss, and EZ loss were graded by reviewing en face SD-OCT slabs for hypo-reflective areas and confirming their status on cross-sectional views. Total area of photoreceptor disruption and overlap with DCP telangiectasia were evaluated with respect to OCT-based MacTel stage. Longitudinal changes were evaluated in a subset of patients with follow-up imaging. Results Overlap of DCP telangiectasia with IZ attenuation significantly decreased with MacTel severity, while overlap with IZ and EZ loss significantly increased. Overlap with IZ loss peaked in moderate MacTel (Stages 3–5). Longitudinal imaging showed that new EZ loss at 6 months was largely predicted by baseline IZ loss. Conclusions Worsening MacTel severity is characterized by greater overlap between DCP telangiectasia and zones of increasing severity of photoreceptor disruption, with EZ loss enlarging over time within areas of preexisting IZ disruption. We suggest that IZ disruption may indicate early photoreceptor dysfunction that eventually progresses to EZ loss, with IZ loss being a more reliable metric than IZ attenuation. Additional studies will be necessary to further explore long-term photoreceptor changes and evaluate their relationship with visual function in MacTel.

Item does not contain fulltext PURPOSE: To study the prevalence and severity of diabetic retinopathy (DR) in patients with macular telangiectasia type 2 (MacTel 2). DESIGN: Retrospective case series. PARTICIPANTS: Patients with a diagnosis of MacTel 2 treated at the Rotterdam Eye Hospital or Erasmus Medical Center between 2014 and 2018 were included. METHODS: The following information was retrieved from patient files: demographics, history of diabetes mellitus and hypertension, presence of DR, and severity of DR, that is, mild, moderate, severe, or proliferative. Presence of diabetic macular edema (DME) was assessed using OCT. MAIN OUTCOME MEASURES: Presence and severity of DR. RESULTS: Two hundred six eyes of 103 patients were included. At the onset of MacTel 2, the mean age was 61 years (standard deviation [SD], 9.8 years) and 64 (62%) were women. Mean follow-up was 71 months (SD, 60 months). Diabetes mellitus type 2 was present in 50 patients (49%) and hypertension was present in 47 patients (46%). Mild DR was present in 22 eyes (11%), of which 14 eyes (7%) showed signs at baseline and 8 eyes (4%) showed signs at a later time during follow-up. Ten eyes (5%) demonstrated remission of mild DR during follow-up. Both eyes (1%) in 1 patient progressed to moderate DR. Severe DR, proliferative DR, and DME did not occur. CONCLUSIONS: Although diabetes mellitus was highly prevalent among MacTel 2 patients, no patients showed severe or proliferative DR or DME. These findings suggest that MacTel 2 could have a protective effect on the progression of DR. We hypothesize that our results may be explained by the role of Müller cells in the development of MacTel 2 and DR, and therefore a link between both diseases warrants additional studies.

PURPOSE Macular telangiectasia (MacTel) Type 2 is a progressing neurovascular disease of the macula, currently lacking effective treatment. This study assessed the effect of nondamaging retinal laser therapy (NRT) compared with sham. METHODS Twelve MacTel patients were enrolled in this double-masked, controlled, randomized clinical trial. For the nine patients with both eyes eligible, one eye was randomized to NRT or sham and the other received alternate treatment. For three patients with only one eye eligible, that eye was randomly assigned either NRT or sham. Ellipsoid zone disruption, best-corrected visual acuity, and macular automated perimetry at 12 months served as structural and functional measures. RESULTS Eleven eyes were randomized to sham and 10 to NRT. Baseline best-corrected visual acuity was 66 letters (20/50) for sham and 72 letters (20/40) for NRT (P = 0.245). Ellipsoid zone disruption area was 298 µm2 in sham and 368 µm2 in NRT (P = 0.391). At 12 months, ellipsoid zone disruption increased by 24% in sham and decreased by 34% in NRT (P < 0.001). Best-corrected visual acuity measures remained stable during follow-up compared with baseline. At 1 year, the mean macular sensitivity was 28 dB in the NRT group, compared with 26 dB in sham. CONCLUSION Nondamaging retinal laser therapy was safe and well tolerated in patients with MacTel and resulted in structural and functional improvements, which could represent a protective effect of laser-induced hyperthermia. Longer follow-up and larger number of patients should help corroborate these effects.

Purpose: To visualize the mode of action of anti-vascular endothelial growth factor (anti-VEGFs) therapy on retinal neovascularization (RNV) in a patient with macular telangiectasia (MacTel) type 2 using a detailed three-dimensional data environment. Observation: A 60-year-old man presented with visual acuity loss and was diagnosed with MacTel type 2. Fluorescein angiography was not possible for safety reasons because of a history of severe reaction to fluorescein dye at his referring hospital. Optical coherence tomography angiography (OCTA) imaging revealed new retinal neovascular membranes (RNV) in the macula of both eyes. A marked reduction in the size of the RNV in both eyes was evident on volume-rendered three-dimensional OCTA retinal imaging after the first anti-VEGF injection. Conclusion and importance: The ability to directly observe the effect of anti-VEGF injections on a RNV using three-dimensional OCTA was successfully demonstrated. This can be useful in patients with previous allergic and potentially lethal complications to fluorescein. In addition, enhanced three-dimensional spatial display of RNV leads to a greater understanding of the perfusion profile and the anatomical changes that occur in ocular neovascularization relative to surrounding tissue. This has the potential to provide insight into the pathobiology of angiogenesis.