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Purpose: To compare AngioTool (AT) vascular parameters (VP) between MacTel2 eyes and normal eyes. Secondary outcome measures were to correlate VP with BCVA and to analyze VP between various grades of Simple MacTel Classification., Methods: This is a retrospective study. SD OCTA images of the superficial vascular complex (SVC) and deep capillary complex (DVC) were exported into Image J and AT. The explant area (EA), vessel area (VA), vessel percentage area (VPA), total number of junctions (TNJ), junction density (JD), total vessel length (TVL), average vessel length (AVL), total number of endpoints (TNE) and mean E lacunarity (MEL) were studied., Results: Group 1 had 120 MacTel2 eyes. Group 2 had 60 age-matched normal eyes. All VP were significantly different between the two groups except EA and TNE in both complexes. None of the VP had a correlation with BCVA. Interquadrant analysis (IQA) in SVC and DVC showed statistical significance in VPA, AVL and JD and in AVL, TNE, JD, VPA respectively. Post hoc analysis in SVC and DVC showed statistical significance in TNJ, JD, TVL and AVL between grade 1 and grade 3, and in VA, VPA, TNJ, JD, TVL and MEL between grade 0 and grade 3 respectively., Conclusion: VP were affected in MacTel2 eyes. VP did not correlate with BCVA. Occurrence of pigmentation is an important event in the progression of disease. AT may provide quantitative markers to measure disease progression., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To describe imaging features of macular telangiectasia type 2 eyes experiencing ellipsoid zone (EZ) recovery., Methods: Macular telangiectasia type 2 patients with EZ recovery were identified from the Natural History and Observational Registry study and underwent retinal imaging including optical coherence tomography and fundus photography. Eyes were graded according to the classification system by Gass and Blodi, the EZ-loss area was measured, and optical coherence tomography parameters were assessed by two independent readers. Parameters were analyzed for their presence before EZ recovery., Results: Twenty-four eyes of 21 patients (12 female, 57.12%; mean age 68 ± 8.54 years) were included in this study and followed for 21.25 months ± 12.79 months. At baseline, mean EZ-loss area was 0.036 mm 2 ± 0.028 mm 2 and 0.01 mm 2 ± 0.013 mm 2 at follow-up ( P < 0.001). A persisting external limiting membrane overlaying the EZ-loss was detected in 16 cases (66%), and hyperreflective changes in the outer retina were present in 18 cases (75%). Best-corrected visual acuity was 0.23 (20/32) ± 0.33 logMAR at baseline and 0.34 (20/40) ± 0.34 logMAR at follow-up ( P = 0.3)., Conclusion: Distinct optical coherence tomography features precede ellipsoid zone recovery in macular telangiectasia type 2 and warrant further studies investigating implications for patient care and clinical trial interpretation.

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 assess visual outcomes of Coats disease by classification stage., Methods: A retrospective review was conducted on consecutive patients with Coats disease, classified according to Shields classification and with available Snellen visual acuity before and after treatment., Results: There were 160 eyes with Coats disease (stage 1 (n=2) vs stage 2A (n=17) vs stage 2B (n=22) vs stage 3A1 (n=26) vs stage 3A2 (n=40) vs stage 3B (n=42) vs stage 4 (n=9) vs stage 5 (n=2)). By comparison, more advanced stage showed greater frequency of poor presenting visual acuity (<20/200) (0% vs 0% vs 50% vs 35% vs 38% vs 83% vs 100% vs 100%, p<0.001) and higher mean intraocular pressure (17 vs 15 vs 15 vs 15 vs 15 vs 15 vs 37 vs 26, p<0.001). More advanced stage was less likely managed with laser photocoagulation (100% vs 87% vs 48% vs 62% vs 74% vs 35% vs 0% vs 0%, p<0.001) and more likely with cryotherapy (0% vs 47% vs 81% vs 81% vs 82% vs 88% vs 50% vs 100%, p=0.001). More advanced stage was associated with lower frequency of visual acuity ≥20/40 (100% vs 71% vs 5% vs 42% vs 23% vs 5% vs 0% vs 0%, p<0.001) and greater frequency of visual acuity <20/200 (0% vs 12% vs 36% vs31 % vs 45% vs 88% vs 0% vs 100%, p<0.001)., Conclusion: Visual acuity in eyes with Coats disease parallels staging with more advanced stage demonstrating poorer visual acuity at presentation and final visit., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To quantify the retinal and choriocapillaris perfusion in different disease stages of macular telangiectasia type 2 (MacTel) using optical coherence tomography-angiography (OCT-A)., Methods: We examined 76 eyes of 76 patients and 24 eyes of 24 age-related controls. Participants underwent multimodal imaging, including OCT and OCT-A. Patients' eyes were divided into three groups considering predefined criteria from funduscopy, OCT, and fluorescein angiography, thus reflecting the disease severity ("early," "advanced," and "neovascular"). Quantitative analyses of vessel density (VD), skeleton density (SD), and fractal dimension (FD) were conducted in the superficial and deep retinal plexus and in the avascular layer. The choriocapillaris was analyzed for mean signal intensity and percentage of nondetectable perfused choriocapillaris-area (PNPA)., Results: The deep retinal plexus showed a progressive decrease of mean VD, SD, and FD in the temporal parafovea in all disease stages. In the superficial layer, VD, SD, and FD were significantly decreased in the temporal parafovea of advanced and neovascular stages, while these parameters did not differ from controls in early stages. In MacTel, signals of blood flow were also detectable at the level of the avascular layer and showed a significant increase with disease progression. The choriocapillaris in MacTel showed a significant increase of mean PNPA and a decrease of mean signal intensity in comparison to controls. These findings were consistent in all disease stages., Conclusions: Quantitative OCT-A data show a progressive rarefication of the retinal microvasculature in MacTel. We propose an altered choriocapillaris perfusion as a possibly early alteration of the disease.

Coats disease is an idiopathic retinal vascular disorder with retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction. The condition is sporadic with no associated systemic abnormalities. Unilateral involvement in young males is the typical presentation with most cases being diagnosed in the first and second decade of life. Younger the patient, more severe is the presentation and poorer the visual outcome. The management varies with the stage of the disease. Over the years, we have shifted from enucleation to a more conservative approach for the treatment of Coats disease with laser photocoagulation, cryotherapy and surgery for retinal detachment achieving good outcomes. The anti-VEGF agents have come into the scene as important form of adjuvant treatment along with the traditional management options. This article describes the clinical features, underlying pathology, classification and staging, the complications and the management of Coats disease and gives an overview of the changing trends in treatment and outcomes spanning across five decades., Competing Interests: None

Purpose: To evaluate the long-term outcomes of treatment of total exudative retinal detachments (ERDs) secondary to Coats disease (stage 3B) and the role of vitrectomy., Design: Retrospective, observational case series., Participants: A total of 16 eyes in 16 patients undergoing treatment for total ERDs secondary to Coats disease with at least 5 years of follow-up., Methods: We reviewed the records of patients with stage 3B Coats disease. The interventions, including the timing of vitrectomy if used, and clinical course were recorded., Main Outcome Measures: The primary outcome measures were visual acuity at the most recent appointment, whether there was progression to neovascular glaucoma (NVG) or phthisis bulbi, and need for enucleation., Results: All patients received ablative treatment (photocoagulation or cryotherapy), with 8 having scleral buckling (SB) and 6 having external drainage of subretinal fluid (XD). Of the 12 patients who had pars plana vitrectomy (PPV), 8 had early PPV (EV) in the first year after presenting, and 4 of 8 in the expectant management group had late PPV (late vitrectomy) at a mean of 4.3 years post-presentation for treatment of significant traction retinal detachment (TRD). The other 4 patients of 8 in the expectant management group did not require vitrectomy. Mean follow-up overall was 9 1/2 years. At the date of last follow-up, 50% had no light perception or light perception vision, which was consistent across the subgroups that underwent EV (4/8), late vitrectomy (2/4), or no PPV (2/4). A total of 4 of 16 patients had progression to NVG or phthisis, 1 of whom required enucleation., Conclusions: In this retrospective series of patients with Stage 3B Coats disease, ablative therapy with a combination of PPV, XD, or SB was effective in preventing progression to NVG or phthisis in the majority of patients, thus preserving the globe. Half of the patients (4/8) in this series who did not undergo PPV in the early vitrectomy group developed late-onset TRD, suggesting a possible role for early prophylactic vitrectomy with possible SB and XD; however, this is balanced by the other half (4/8) in the expectant management group who did not require any vitrectomy., (Copyright © 2018 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: Type 2 idiopathic macular telangiectasia (MacTel2) is a rare retinal disease that has still not been well-defined. The aim of the present manuscript is to describe the clinical features by multimodal retinal imaging, to present the functional characteristics, and to estimate the prevalence of the disease., Methods: A retrospective study was conducted on the 12 eyes of 6 patients with MacTel2. Fundus colour photographs, fundus autofluorescence, fluorescein angiography, optical coherence tomography (OCT), and OCT-angiography were performed and subsequently analysed. Visual acuity (VA) was prospectively recorded. The prevalence was established based on the patients referred to a specialised macular diseases unit. Minimum follow-up period was 18 months., Results: Prevalence of MacTel 2 in our study was 0.12%. Clinical features were presented using multimodal retinal imaging. VA remained stable during follow-up. Three patients developed choroidal neovascularisation (CNV), requiring intravitreal treatment with antiangiogenic agents., Conclusions: The prevalence of the disease found was 0.12%. The study using multimodal imaging allows a more accurate diagnosis and follow-up of this pathology. The VA is maintained during the 18-month follow-up (P=.492). Patients who develop CNV and are treated with antiangiogenic agents appear to respond adequately to them. More studies are needed to establish these conclusions., (Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Purpose: To evaluate changes in foveal and parafoveal thickness measured using optical coherence tomography across five stages of macular telangiectasia 2 (MacTel 2) and to correlate the foveal slope angle with best-corrected visual acuity (BCVA)., Methods: A multicentered, cross-sectional study of 90 patients with bilateral MacTel 2 was carried out. We reviewed medical records of patients who had undergone optical coherence tomography. Mean retinal thickness foveal and subfoveal were noted from ETDRS (Early Treatment Diabetic Retinopathy Study) mapping and later used for calculating foveal slope angle. The change in retinal thickness and foveal angle across five stages of MacTel 2 was assessed. Central foveal thickness and foveal slope angle were correlated with BCVA., Results: The foveal slope angles were calculated quadrantwise and stagewise. The mean slope angle was minimum in temporal quadrant (6.29°) followed by inferior (7.13°), superior (7.54°), and nasal (7.93°). The slope angles were 9.27°, 7.95°, 6.70°, 6.10°, and 6.31° in Stages 1, 2, 3, 4, and 5, respectively. Statistically significant correlation noted between the temporal, superior, inferior slope angles and BCVA (in logarithm of the minimum angle of resolution) with r = -0.18, -0.19, and -0.25, respectively (P < 0.05). No statistically significant correlation was noted between central retinal thickness and BCVA., Conclusion: The BCVA correlates better with slope angles than central retinal thickness. This implies that, change in both foveal and parafoveal regions accounts for vision change. Greater the foveal slope angle, the better the vision.

Purpose: To determine the prevalence, clinical characteristics and nature of subfoveal nodules in Coats' disease and the associated impact on the long-term visual outcome., Methods: Consecutive cases of Coats' disease with foveal exudation were retrospectively reviewed. The presence of a subfoveal nodule or macular fibrosis was recorded. Clinical characteristics, retinal imaging, and outcome were analyzed by comparative analysis. The histopathological description of an enucleated eye with subfoveal nodule was performed., Results: Among 40 patients presenting unilateral Stage 2B or 3A1 Coats' disease, a subfoveal nodule was detected in 21 patients (52.5%). The median follow-up was 4.7 years. Nineteen patients (47.5%) did not present a subfoveal nodule. Three patients (15.8%) without subfoveal nodule and 21 patients (100%) with subfoveal nodule progressed to a macular fibrotic scar (P < 0.0001), and the mean time of macular fibrosis onset was 11.0 ± 2.6 months. Final visual acuity was significantly worse in patients who presented a subfoveal nodule at diagnosis (P = 0.01). Of 18 cases with subfoveal nodule who underwent fluorescein angiography, retinal-retinal anastomosis and neovascularization were detected in 13 (72.2%) and 2 eyes (11.1%), respectively. Histopathological analysis of a subfoveal nodule revealed an aggregate of proteinaceous material including fibrin, spindle cells, macrophages, and pigmented cells., Conclusion: The presence of a subfoveal nodule at presentation is a predictive factor for macular fibrosis development and worse visual outcome in patients with Coats' disease. These observations suggest an updated classification introducing two subcategories within Stage 2B: without subfoveal nodule (Stage 2B1) and with subfoveal nodule (Stage 2B2).

Purpose: To describe the outcomes of children with Coats disease treated with 577-nm yellow laser indirect ophthalmoscopy., Methods: A retrospective consecutive case series of pediatric patients with Coats disease treated at a single institution between 2011 and 2014. Laser indirect ophthalmoscopy was performed under anesthesia. Full treatment was defined as complete ablation of all visible telangiectasias and resolution of subretinal fluid. No patients were treated with cryotherapy or bevacizumab., Results: Seventeen eyes of 16 patients were consecutively treated. At the time of diagnosis, the eye was classified as Stage 1 (telangiectasias only) in 1 case, Stage 2A (extra-foveal exudation) in 2 cases, Stage 2B (fovea-involving exudation) in 6 cases, Stage 3A1 (extra-foveal exudative retinal detachment) in 2 cases, Stage 3A2 (subtotal foveal-involving detachment) in 1 case, and Stage 3B (total exudative retinal detachment) in 5 cases. The mean age at initial treatment was 71.2 months. Mean length of follow-up was 20.8 months (median 18.5 months, range 3.7-37.3 months). Patients underwent an average of 2.5 laser treatments. Sixteen of 17 eyes achieved full treatment (94.1%) with a mean time-to-full-treatment of 11.2 months. One eye developed glaucoma and end-stage disease., Conclusion: 577-nm yellow wavelength laser indirect ophthalmoscopy is an effective treatment for Coats disease including cases of exudative retinal detachment.

Purpose: To review the surgical outcomes of intravitreal bevacizumab (IVB) along with subretinal fluid drainage with cryotherapy in patients with stage 3B Coats' disease., Materials and Methods: A retrospective study of seven cases of stage 3B Coats' disease, who underwent subretinal fluid drainage with cryopexy, from May 2011 to March 2014. Five eyes received additional IVB at the end of surgery. Green laser therapy was performed on telangiectatic vessels postoperatively., Results: The mean age was 34 months (range, 10-84 months). Mean follow-up was 19 months. Six patients (85.7%) had an attached retina at final follow-up. Three out of four patients (75%) that received IVB developed tractional retinal detachments (TRDs). Two eyes that did not receive bevacizumab did not develop any traction. None progressed to neovascular glaucoma or phthisis bulbi., Conclusion: Simultaneous injection of bevacizumab along with subretinal drainage and cryotherapy for advanced Coats' disease could not avoid TRD.

Background: To report very early morphologic and functional alterations in patients with macular telangiectasia type 2., Methods: Patients with asymmetric disease manifestations, in whom retinal alterations characteristic for macular telangiectasia type 2 were present in one but not in the apparently unaffected fellow eye, underwent multimodal imaging and functional testing (microperimetry, visual acuity, reading ability, Amsler test)., Results: Fellow eyes not allowing the diagnosis of macular telangiectasia type 2 based on hitherto diagnostic standards consistently showed a severely reduced directional cone reflectance (Stiles-Crawford effect). Optical coherence tomography revealed an asymmetric configuration of the foveal pit with focal temporal thinning most pronounced at 1° eccentricity. Topographically related, macular pigment optical density was reduced in a small wedge-shaped temporal paracentral sector, resulting in an increased signal on fundus autofluorescence and fluorescein angiography imaging. No functional deficits were detectable in fellow eyes. Haidinger brushes were perceived in the fellow eye but not in the affected index eye with pronounced loss of macular pigment., Conclusion: Specific morphologic alterations precede vascular alterations and functional deficits in macular telangiectasia type 2. The described alterations indicate a primarily degenerative process with a secondary retinal vascular phenotype, and may be helpful for early identification of patients and affected family members.

Purpose: To determine whether cone photoreceptors are impaired early in macular telangiectasia type 2 (MacTel 2) progression., Methods: Eight patients with MacTel 2 lacking intraretinal cavitation underwent multimodal retinal imaging including adaptive optics. Cone packing metrics were determined in 5 sampling windows at different eccentricities from the fovea. Results were compared with a previously established normative database., Results: In MacTel 2 eyes, cone density was significantly lower than normal at all eccentricities (P < 0.0001). Mean cone spacing and mean percentage of hexagonally organized cone photoreceptors were respectively significantly larger and lower than normal at all eccentricities (P = 0.0488 and P < 0.0001). In MacTel 2 patients, adaptive optics showed an irregular patchy disturbance of the cone mosaic corresponding to some fragmentation of the interdigitation zone on optical coherence tomography. The ellipsoid zone remained intact in the studied area., Conclusion: Adaptive optics showed that the macular cone density was lower than normal even outside the telangiectasia in MacTel 2 lacking intraretinal cavitation, although the ellipsoid zone remained intact on optical coherence tomography. These findings do not indicate that the cone density loss is causative of the disease as it might be secondary to Müller cell or rod loss in this area. However, cone density assessment could become a useful parameter to monitor disease progression.

Macular telangiectasia type 2 (MacTel2) is a disease of the central macula that affects all microvascular layers of the retina and also includes neovascularization arising from both the retinal and choroidal circulations. As a result, MacTel2 is the ideal macular disease for imaging with optical coherence tomography (OCT) angiography (OCTA). In MacTel2, the earliest changes in the retinal microvasculature involve the temporal aspect of the parafoveal deep capillary plexus, and OCTA reveals these changes. Microvascular abnormalities then extend circumferentially around the fovea and into the superficial capillary plexus. As the disease progresses, dilated anastomoses form between the superficial and deep capillary plexuses, and the retina becomes more atrophic, with the formation of cysts and the loss of photoreceptor outer segments. In some patients, the anastomoses between the plexuses progress to subretinal neovascularization, with connections to the choroidal vasculature. OCTA provides clear advantages over current imaging modalities, such as traditional OCT imaging, reflectance fundus imaging, autofluorescence imaging, fluorescein angiography, and indocyanine green angiography, for MacTel2 because it is safer, cheaper, more comfortable for the patient, and more easily repeatable, it can be performed during follow-up visits, and it produces both intensity-based OCT images and flow-based images, which allow for visualization of the macular microvasculature. OCTA is the only imaging modality needed for the diagnosis and monitoring of MacTel2 at every stage of disease progression., (© 2016 S. Karger AG, Basel.)

Background: To evaluate the effectiveness of endolaser photocoagulation by a two-port pars plana nonvitrectomy approach for treating Coats' disease with shallow exudative retinal detachment., Methods: This study included 24 patients (23 boys with an age range of 2-17 years, and one girl, age 6 years) with stage 3 Coats' disease (25 eyes) from December 2012 and May 2014 at a single center. All of the 25 eyes were complicated with serous or total retinal detachment and received none-vitrectomized endolaser: two (23- or 25-gauge) incisions were routinely made 3 mm posterior to the corneal limbus and a laser was applied directly on the abnormal blood vessels. Additional treatments included subretinal fluid drainage (five eyes), intravitreal triamcinolone injection (seven eyes), and intravitreal anti-vascular endothelial growth factor (VEGF) injection (17 eyes). Best-corrected visual acuity, intraocular pressure, and fundus and abnormal vascular changes were recorded to determine therapeutic effects., Results: Twenty-four out of the 25 treated eyes (96 %) had retina reattached. The number of treatment sessions differed case by case (1-5 sessions, average 1.96) and the time to full treatment of retinal reattachment was 4 months in average. One patient (4 %) presented with retinal redetachment. Five (20 %) eyes received further laser treatment with indirect ophthalmoscope and four eyes (16 %) presented with total retinal detachment at their first visits received consecutive treatments. At the end of the follow-up period (mean, 10.08 months), telangiectasias of 24 (96 %) eyes were resolved and no severe complications occurred., Conclusions: Endolaser photocoagulation by a two-port pars plana nonvitrectomy approach is an effective treatment for advanced Coats' disease with serous retinal detachment. The long-term safety of the approach needs further investigation.

Purpose: To investigate progressive vision loss in patients with macular telangiectasia (MacTel) type 2 and to compare the ability to detect functional decline between microperimetry and visual acuity testing., Methods: Change of cumulative defect size (number of test points with absolute scotoma) on microperimetry testing and change in distance best-corrected visual acuity (BCVA) were evaluated in a prospective longitudinal observational study., Results: The mean review period was 55.3 months (SD 17.3 months). In 58% of 71 eyes (40 patients) included for analysis, microperimetry revealed spread (n = 31) or new development (n = 10) of an absolute scotoma. At the same time, BCVA decreased more than two lines in only 17% (n = 12). Twenty-five (35%) eyes showed no change in visual function. Presence of an absolute scotoma at baseline, but not baseline BCVA, was predictive for functional decline on longitudinal microperimetry testing. Eyes with an absolute scotoma at baseline (n = 33) showed further growth of the scotoma in 94% (n = 31). In contrast, only 26% (n = 10) of eyes without an absolute scotoma at baseline (n = 38) developed an absolute scotoma de novo. Scotoma growth rate (new test points with an absolute scotoma per year) was 0.62 ± 0.10 for all eyes and 1.30 ± 0.12 for the subgroup of eyes with scotoma at baseline. Scotomata always first occurred in the quadrant temporal to the foveal center., Conclusions: A characteristic feature in patients with MacTel type 2 is progressive focal loss of macular sensitivity, preceding loss of visual acuity. Microperimetry is sensitive to detect such functional decline and thus may provide considerable power when being used as a functional outcome measure in future clinical trials.

Purpose: To review the anatomic and visual outcomes of a series of children diagnosed with Coats' disease and treated on the basis of intraoperative fluorescein angiography (FA) findings., Design: Retrospective case series., Participants: Twenty-five children 2 to 15 years of age diagnosed with early Coats' disease and treated after intraoperative FA., Methods: Retrospective review of patients who underwent treatment for stage 2 Coats' disease in a tertiary center in the United Kingdom between 2007 and 2012. The children underwent treatment to the telangiectatic vessels and associated areas of retinal nonperfusion identified on intraoperative FA performed with a wide-angle retinal camera (RetCam II)., Main Outcome Measures: Anatomic and structural assessment of the retina after treatment, visual acuity (VA), and complications related to treatment., Results: Twenty children with Coats' disease who underwent intraoperative RetCam FA and retinal ablative treatment and who had more than 3 months of follow-up were identified from clinical records. All had unilateral disease. Six eyes were classified as stage 2a, and 14 eyes were classified as stage 2b. The median duration of follow-up was 21 months (mean, 26 months). Fifteen eyes needed 1 treatment to stabilize the disease, and 5 eyes needed a second treatment. None of the patients demonstrated progression of the disease to a more severe stage. Twelve eyes had a final VA of 0.4 logarithm of the minimum angle of resolution (logMAR) or better, 6 patients had VA between 0.4 and 1.0 logMAR, and 2 patients had VA worse than 1.0 logMAR., Conclusions: The treatment of retinal telangiectasia and areas of retinal nonperfusion identified by wide-angle intraoperative FA in children with stage 2 Coats' disease led to good anatomic outcome, with preservation of VA in most cases., (Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background and Objective: To evaluate subfoveal choroidal thickness (CT) and the extent of outer retinal disruption in patients with macular telangiectasia type 2 (MacTel2) compared with healthy eyes., Patients and Methods: In this prospective, observational, cohort study, 62 patients (62 eyes) with Mac-Tel2 and 130 healthy controls (130 eyes) underwent a complete ophthalmological examination, spectral-domain optical coherence tomography (SD-OCT) imaging, and axial length measurements. Patients in the study group also underwent color fundus photography, fundus autofluorescence, and fluorescein angiography. En face SD-OCT imaging was used to assess abnormalities involving the photoreceptor inner segment/outer segment/ellipsoid zone (IS/OS/EZ)., Results: After adjusting for age and axial length, the authors found that eyes with MacTel2 had a mean CT measurement that was greater than control eyes (P = .007). There was a negative correlation between the visual acuity and the area of IS/OS/EZ damage (P = .009), but no statistically significant correlation was seen between CT and the area of IS/OS/EZ damage., Conclusion: Eyes with MacTel2 were found to have thicker CT measurements than control eyes. While the extent of IS/OS/EZ disruption correlated with the loss of visual acuity, this damage did not correlate with CT measurements., (Copyright 2015, SLACK Incorporated.)

The objective of the study is to report, and propose a hypothesis for, a novel clinical sign in patients with macular telangiectasia type 2. Nine consecutive patients with macular telangiectasia and visible parafoveal graying were examined. The parafoveal graying decreased in intensity with continuous light exposure from an indirect ophthalmoscope. After dark adaptation for 15 minutes, the intensity of parafoveal graying increased again. This phenomenon appears to be a novel and global sign in patients with macular telangiectasia type 2. It could be a possible photochemical reaction occurring due to the release of chromophores from the abnormal Müller cells., (Copyright 2015, SLACK Incorporated.)

Purpose: To comprehensively evaluate the retinal and choroidal changes in eyes with Type 2 idiopathic macular telangiectasia using "en face" and B-scan spectral domain optical coherence tomography (OCT), and to compare their respective contributions to this evaluation., Methods: Eyes with a diagnosis of proliferative or nonproliferative Type 2 macular telangiectasia were prospectively studied. All patients underwent an extensive ophthalmologic examination including biomicroscopic fundus examination, color photography, fundus autofluorescence, fluorescein angiography, B-scan and en face spectral domain OCT., Results: Twenty eyes of 10 patients were included in this study. En face OCT C-scans and conventional B-scans were both able to show inner crystalline deposits (15%), retinal capillary anomalies (100%), intraretinal cysts (80%), hyperreflective spots in the outer nuclear layer (100%) and external limiting membrane (80%), hyperplastic pigment plaques (30%), intraretinal neovascularization (20%), photoreceptor loss (100%), and choroidal cavitations (30%). En face OCT C-scans provided more information than B-scans on intraretinal neovascularization, photoreceptor loss, and choroidal cavitations. Also, en face OCT C-scans provided better visualization of the retinal vessels and telangiectasia than fluorescein angiography., Conclusion: En face OCT is a noninvasive and reproducible technique that helps to better assess and follow up retinal and choroidal processes in Type 2 macular telangiectasia.

Purpose: To quantitatively analyze the distribution of macular pigment (MP) over a period of 5 years and for monitoring progression of macular telangiectasia., Methods: Macular pigment concentration (autofluorescence, excitation wavelengths: 488 and 514 nm) was determined at baseline and after 5 years in 43 eyes of 22 subjects (46-80 years; mean, 65.6 years; 10 men) participating in the macular telangiectasia project., Results: Mean MP density at 0.5° declined in the segment (one eighth of a circle) with the highest MP optical density (-0.04 density units; P= 0.015), where density units (DU), and also averaged in the 2 segments that divided segments with detectable MP from those in which MP was no longer detectable (-0.04 density units; P = 0.0005). In the first segment mentioned, 2° values decreased to a lesser extent and not significantly. The diameter of MP loss expanded horizontally from 2.64 mm to 2.74 mm (P = 0.0001) but not vertically. Macular pigment density in the "halo" of peripheral MP at a mean of 5.44° (4.53-6.21°) increased (+0.01 DU; P= 0.01)., Conclusion: Five years of follow-up resulted in central (0.5°) reduction and peripheral (4.53-6.21°) accumulation of MP. Longer period of follow-up may disclose significant changes in paracentral locations. The area of central MP loss expands in particular in a horizontal direction and less vertically. Centrifugal movement of MP during disease may explain our findings.

Macular telangiectasia type 2-also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A or Mac Tel 2-is an acquired bilateral neurodegenerative macular disease that usually manifests itself during the fourth to sixth decades of life and is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right-angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV). Optical coherence tomography images typically demonstrate intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic finding is a deep intraretinal hyperfluorescent leakage in the temporal parafoveal area. With time the leakage may involve the whole parafovea, but does not extend to the center of the fovea. Long-term prognosis for central vision is variable and depends on the development of SRNV or macular atrophy. Pathogenesis remains unclear, but Müller cells and macular pigment appear to play a central role. Currently there is no known treatment for the underlying cause of this condition, but treatment of the SRNV may be beneficial., (Copyright © 2013 Elsevier Inc. All rights reserved.)

The clinical and imaging findings and therapeutic outcomes of intravitreal bevacizumab injection in a patient with macular telangiectasia type 2 are described. The patient first presented with the non-proliferative stage of the disease for 4 months, then the disease transformed to the proliferative stage. In the proliferative period, the patient was treated with intravitreal bevacizumab injections as-clinically warranted. Over a follow up period lasting 26 months, the patient received 6 intravitreal bevacizumab injections, the visual acuity improved from 20/100 to 20/40, the central retinal thickness decreased from 318 microns to 198 microns. This case implies that the patients with non-proliferative macular telangiectasia type 2 should be followed carefully for proliferative transformation, and intravitreal bevacizumab treatment seems to be effective for proliferative macular telangiectasia type 2.

Purpose: To compare pathologic changes in the photoreceptors of eyes with idiopathic macular telangiectasia types 1 and 2 using adaptive optics scanning laser ophthalmoscopy (AO-SLO)., Design: Prospective cross-sectional study., Methods: Eleven eyes with idiopathic macular telangiectasia type 1, 14 eyes with type 2, and 10 normal eyes underwent a full ophthalmologic examination, spectral-domain optical coherence tomography (SD-OCT), and imaging with an original prototype AO-SLO system. All eyes with idiopathic macular telangiectasia were examined with fluorescein angiography (FA), confocal blue reflectance, and microperimetry., Results: Compared with normal eyes (29 017 ± 5507 cones/mm(2)), those with idiopathic macular telangiectasia type 1 had significantly lower cone density on the temporal side (18 427 ± 4908 cones/mm(2), P = .010). The FA leakage area (5.90 ± 3.23 mm(2)) was larger than the dark regions (2.45 ± 2.40 mm(2)) on AO-SLO (P = .003)-which were observed only in the FA leakage area. Compared with normal eyes, those with idiopathic macular telangiectasia type 2 had significantly lower cone density in all areas (P < .05 for all). Patchy dark regions were also seen in areas without FA abnormalities in 12 eyes. Higher mean retinal sensitivity correlated with greater mean cone density 0.5 mm from the center of the fovea (type 1: P = .010; type 2: P < .001)., Conclusions: Idiopathic macular telangiectasia type-specific differences in the distribution of photoreceptor abnormalities were shown in the AO-SLO images. For both idiopathic macular telangiectasia types 1 and 2, visual impairment was associated with cone damage., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: To characterize the electroretinographic response of the macula by multifocal electroretinography (mfERG) in patients with type 2 idiopathic macular telangiectasia (MacTel)., Methods: A prospective study of mfERG in patients with type 2 MacTel was conducted from April 2009 to November 2009. mfERGs were recorded using a visual evoked response imaging system (MonElec2, Metrovision, Perenchies, France). The International Society for Clinical Electrophysiology of Vision (ISCEV) guidelines were followed. Patients with type 2 MacTel confirmed by fundus fluorescein angiography without subretinal neovascularisation were included. For recording purposes, 61 stimulus hexagonal elements were used. The first-order kernel mfERG responses were analyzed. Individual mfERG responses for the hexagons were grouped into concentric rings centered on the fovea for analysis (< 2, 5-10, 10-15 and >15°). Student's t-test and Mann-Whitney U test and linear regression analysis was performed with STATA ver 11.1 (StataCorp, College Station , TX, USA)., Results: Twenty eight eyes of 14 patients and 20 eyes of ten normal controls were included in the study. The mean logMAR visual acuity of the patients was 0.51 (Snellen equivalent 20/63). The mean N1 amplitude (nv/deg(2)) of patients were significantly reduced compared to controls and were as follows: 8.91 ± 14.00 vs 43.44 ± 9.55 (p < 0.0001) in less than 2°, 9.24 ± 10.47 vs 22.00 ± 3.87 (p < 0.0001) in 5-10°, 8.57 ± 10.02 vs 15.24 ± 1.89 (p < 0.0001) in 10-15°, and 7.03 ± 6.52 vs 12.47 ± 2.62 in > 15° (p < 0.001). The mean P1 amplitude (nv/deg(2)) was also significantly reduced in patients compared to controls and was as follows: 27.66 ± 37.44 vs 96.20 ± 12.41 (p < 0.0001) in less than 2°, 22.61 ± 19.38 vs 53.78 ± 9.79 (p < 0.0001) in 5-10°, 18.75 ± 20.21 vs 35.22 ± 4.16 (p < 0.001) in 10-15°, and 17.10 ± 12.54 vs 25.71 ± 3.93 (p < 0.001). The implicit time of N1 and P1 were also delayed significantly in all the rings. The mean central foveal thickness assessed by optical coherence tomography (OCT) scan was 84.78 ± 45.12 μm. There was poor correlation between mfERG amplitudes or implicit times with either the visual acuity or OCT central thickness., Conclusion: mfERG showed significant reduction in amplitudes and implicit times of the waveforms in patients with type 2 MacTel in all the rings, suggesting a more generalized affection of the macula. The maximum reductions were seen in the <2(o) rings. Although there was poor correlation between the visual acuity and the amplitudes a of the waveforms, mfERG is a useful investigative modality for functional assessment of macula in type 2 MacTel patients.

Purpose: To compare macular pigment in three types of macular telangiectasia (MacTel)., Methods: Twenty-six eyes of 16 patients (mean age, 62 years) with MacTel were examined. The macular pigment optical density (MPOD) was obtained by autofluorescence spectrometry using two wavelengths (488 nm, 514 nm). The density of the central macular pigment within 0.5 degree around the foveal center (MPODc) was measured from a MPOD map., Results: Eight eyes of seven patients were classified as type 1, 16 eyes of eight patients as type 2, and two eyes of one patient as type 3. The macular pigment signal was seen in the area of fluorescein leakage in type 1 and the occlusive area in type 3; the macular pigment signal decreased in the area of fluorescein leakage in type 2. The mean MPODc (± standard deviation) was 0.40 ± 0.16 density unit (DU) (range, 0.17-0.57) in type 1; -0.08 ± 0.15 DU (range, -0.29-0.25) in type 2; and 0.32 ± 0.08 DU (range, 0.26-0.37) in type 3. Significant differences were seen between types 1 and 2 (P < 0.001) and types 2 and 3 (P = 0.007). There was no significant difference between types 1 and 3 (P = 0.765). In 30 age-matched normal eyes, the MPODc was 0.58 ± 0.11 DU (range, 0.38-0.79). There were significant differences between normal eyes and each MacTel subtype (type 1, P = 0.007; type 2, P < 0.001; type 3, P = 0.043)., Conclusions: The macular pigment values and distributions, including leakage or occlusion, differed in each MacTel type compared with fluorescein angiography findings. The capillary occlusion in type 3 is not associated with reduced MPOD.

Purpose: To report unusual presentations of type 2A idiopathic macular telangiectasia (IMT)., Methods: A retrospective analysis of disease presentation was conducted in 32 patients with type 2A IMT. All patients underwent a complete ophthalmological examination, including spectral domain optical coherence tomography (SD-OCT), fluorescein angiography (FA) and indocyanine green angiography (ICGA)., Results: Three out of 32 study patients showed the simultaneous presentation of type 2 IMT and other retinal diseases. In the first patient, the ophthalmological examination revealed a proliferative IMT associated with late-onset Stargardt disease. The second patient presented bilateral nonproliferative IMT and chronic serous chorioretinopathy in the left eye. The examination of the third patient revealed basal laminar drusen and soft drusen associated with IMT in both eyes., Conclusions: Type 2 IMT may represent an unusual presentation of Stargardt disease, chronic serous chorioretinopathy and basal laminar drusen. These presentations are most likely coincidental and highlight the importance of FA, ICGA and SD-OCT in the diagnosis and treatment of such cases., (© 2013 S. Karger AG, Basel.)

Background/aims: The aim of this paper is to report the treatment of type 2 nonproliferative idiopathic macular telangiectasia (IMT) with intravitreal bevacizumab (IVB)., Methods: Retrospective case series of 10 eyes of 5 patients with type 2 IMT. All patients received 3 monthly IVB injections. Visual acuity (VA), fluorescein angiography (FA) and optical coherence tomography (OCT) were performed at baseline and 4 weeks after each injection., Results: Four weeks after the third IVB injection, VA remained stable for all patients. All eyes showed some decrease in fluorescein leakage, and there was a mild decrease in central macular thickness. One year later, VA, OCT and FA findings returned to the baseline levels., Conclusion: IVB did not improve VA in cases of type 2 IMT., (Copyright © 2013 S. Karger AG, Basel.)

Purpose: Type 2 idiopathic macular telangiectasia (MacTel) is a progressive retinal disease associated with a slow deterioration of visual acuity, starting in the fifth to seventh decades of life. The etiology and pathogenesis of the disease are little known, and no effective therapy is available. We aimed to estimate the prevalence and describe the phenotype of type 2 MacTel in two African populations., Methods: From two population-based cross-sectional surveys conducted nationally in Nigeria and in the Nakuru district of Kenya, patients with fundus features of type 2 MacTel were selected. Diagnosis was based on color fundus images, grading performed according to the MacTel Study protocol and staged using the Gass and Blodi system. Disease phenotype and clinical characteristics of affected participants were assessed., Results: Of 8599 total participants, five showed a phenotype compatible with type 2 MacTel. Prevalence was estimated as 0.06% (95% confidence interval [CI] 0.02-0.21%) in Kenya, 0.06% (95% CI 0.01-0.17%) in Nigeria, and overall at 0.06% (95% CI 0.02-0.14%). Mean age was 62 years (SD 5 years), four of five affected participants were female, and none had a history of diabetes. Median corrected visual acuity was 6/12 in the better eye and 6/69 in the worse eye., Conclusions: The estimated prevalence and phenotype of type 2 MacTel in the African populations examined were similar to those in predominantly white populations. All data published so far are based on the analysis of color fundus images only and are thus likely to underestimate the true prevalence of this disease.

Purpose: To describe fundus autofluorescence (FAF) patterns in patients with type 2A idiopathic juxtafoveolar retinal telangiectasia (IJRT)., Methods: We reviewed FAF images, color photographs, and fluorescein angiography (FA) images of 30 eyes from 16 patients with type 2A IJRT. Eyes with presence of subretinal neovascularization or any other retinal pathology were excluded. All the imaging modalities were obtained with a Heidelberg Retina Angiograph (HRA) confocal laser scanning system., Results: The mean age of the patients was 50.7 years, 68.5% female. At baseline, the median best-corrected visual acuity was 20/50. Loss of normal foveal hypoautofluorescence was noted in 93.3% of eyes. All the eyes showed hypoautofluorescence corresponding to intraretinal crystals and pigment clumps. Increased FAF around the pigments was noted in 93.3% of eyes. Increased FAF corresponding to the angiographic leakage from telangiectatic and nontelangiectatic areas was noted in 86.6% of eyes and 80% of eyes, respectively., Conclusions: Loss of foveal hypoautofluorescence and increased FAF corresponding to the nontelangiectatic angiographic leakage areas were predominant features. Fundus autofluorescence patterns corresponding to color photography and FA findings may further add to the understanding of morphologic alterations in type 2A nonproliferative IJRT at early stages.

Purpose: To describe the natural course of Type 2 idiopathic macular telangiectasia in terms of visual outcomes, causes of visual loss, and incidence of subretinal neovascular membranes (SRNV)., Methods: This retrospective observational case series consisted of chart review of 104 outpatients (203 eyes; 66 women, 38 men) who were diagnosed to have Type 2 idiopathic macular telangiectasia by clinical examination and fluorescein angiography between January 2000 and December 2008. Visual and anatomic outcomes were analyzed during a minimum follow-up of 1 year., Results: The mean age of the patients was 57 years (range, 40-74 years). Nineteen eyes (18 patients) presented with SRNV; the number increased to 29 eyes (14%; 23 patients) by the final visit (mean follow-up, 31 months). Diabetes was common (59%) though retinopathy was initially absent or mild to moderate in 99% patients. Mean logarithm of the minimum angle of resolution best-corrected visual acuity declined from 0.35 to 0.43 by the last visit (P < 0.0001) overall; final mean logarithm of the minimum angle of resolution best-corrected visual acuity was 0.61 (20/80) in the eyes with SRNV and 0.40 (20/50) in eyes without SRNV. The latter group started with better best-corrected visual acuity than SRNV group and remained better at 1-year, 2-year, and final follow-ups (P ≤ 0.0002). Overall, 30 eyes (15%; 24 patients) lost ≥ 2 Snellen lines, the main causes being SRNV and intraretinal pigment migration. Of 128 eyes (including SRNV) with best-corrected visual acuity ≥ 20/40 at baseline, 98 (77%) retained stable visual status; 74 (71%) patients retained best-corrected visual acuity of 20/40 or better at least in 1 eye., Conclusion: Over a follow-up of approximately 3 years, most eyes with Type 2 idiopathic macular telangiectasia starting with good vision were found to retain status quo; sight-threatening complications developed in a minority of eyes; most patients retained good vision at least in 1 eye.

Purpose: Idiopathic juxtafoveal telangiectasia (IJT) is characteristically associated with pigmentary changes. Polarization-sensitive spectral-domain optical coherence tomography (PS-SD-OCT) enables imaging of the retinal pigment epithelium (RPE) and similar melanin-containing structures based on specific polarization properties. This study examined IJT with the latest-generation SD-OCT and PS-SD-OCT, identifying pathophysiologically relevant characteristics of the retinal layers and RPE., Methods: Twenty-two eyes of 12 patients with IJT were examined by PS-SD-OCT, with special focus on RPE detection and segmentation. Furthermore, SD-OCT technology (Cirrus, Spectralis, and 3D-OCT-1000) was applied. Characteristics of the retinal layers and RPE were evaluated. A classification system based on OCT characteristics of IJT was suggested., Results: Polarization-sensitive spectral-domain optical coherence tomography together with SD-OCT identified characteristic patterns of IJT, used to classify eyes into three distinct groups. Group 1 (5 eyes) revealed discrete alterations in the inner retinal layers; group 2 (12 eyes) showed irregularities of the junction between the inner and outer photoreceptor segments with outer retinal atrophy but an intact RPE. Group 3 (5 eyes) revealed RPE irregularities and loss in addition to intraretinal alterations and photoreceptor abnormalities., Conclusion: This study described characteristic morphologic changes in IJT based on PS-SD-OCT and SD-OCT. Morphologic changes were classified, possibly leading to an OCT-based grading scheme. The intensity images of SD-OCT verified intraretinal and photoreceptor irregularities in great detail, whereas PS-SD-OCT additionally showed RPE alterations.

Objectives: To review the frequency and nature of idiopathic macular telangiectasia and to classify the disorders based on new clinical and imaging observations., Methods: A combined retrospective and prospective analysis of newly diagnosed patients seen over a period of 3 years. Patients were identified based on the Gass-Blodi classification and were studied with biomicroscopy, fluorescein angiography, and optical coherence tomography., Results: Ten patients associated with aneurysmal telangiectasia (Gass-Blodi group 1) and 26 patients with perifoveal telangiectasia (Gass-Blodi group 2) were recruited. None with occlusive telangiectasia (Gass-Blodi group 3) were identified. New observations based on clinical, fluorescein angiographic, and optical coherence tomographic findings were made., Conclusions: Our series was similar to that in the Gass-Blodi study in terms of frequency. New observations in groups 1 and 2 have expanded our knowledge of the clinical spectrum of these disorders. A simplified classification termed idiopathic macular telangiectasia with 2 distinct types (type I, or aneurysmal telangiectasia, and type II, or perifoveal telangiectasia) was proposed to produce a better understanding of the entities and to enhance teaching and research. The third type, occlusive telangiectasia, has been omitted from our classification based on its rarity and presence of capillary nonperfusion rather than macular telangiectasia as the primary abnormality.

Purpose: To characterize the phenotype and investigate the associations of intraretinal crystalline deposits in a large cohort with type 2 idiopathic macular telangiectasia (MacTel)., Design: Case-control study., Participants: Patients with and without retinal crystals from the Macular Telangiectasia Project, an international multicenter prospective study of type 2 MacTel., Methods: Grading of stereoscopic 30-degree color fundus (CF), confocal blue light reflectance (CBR), red-free (RF), and infrared (IR) images was performed according to the MacTel Natural History Study protocol and staged using the classification system devised by Gass and Blodi. Spectral domain-optical coherence tomography (SD-OCT) and adaptive optics imaging were used for a finer analysis of the phenotype. Associations between crystals and other characteristics of the disease, as well as potential risk factors, were investigated., Main Outcome Measures: Presence of crystals, fundus signs of MacTel, clinical characteristics, and presence of potential risk factors of MacTel., Results: Of 443 probands enrolled in the MacTel study, 203 (46%) had crystalline deposits present; 60% of the cases were bilateral at baseline. Eyes with crystals had a mean letter score of 70.7 (standard deviation [SD] = 15.9), whereas those without crystals had a mean letter score of 66.5 (SD = 15.5, P < 0.001). Crystals were present at all stages of the disease and showed high reflectivity within a wide wavelength range. They were located at the anterior surface of the nerve fiber layer, arranged along the nerve fibers, within an annular area centered on the fovea. Significant associations of crystalline deposits were found with a loss of retinal transparency, macular pigment optical density (MPOD) loss, fluorescein leakage, retinal thickness, and a break in the inner segment/outer segment junction line. Associations with environmental risk factors were not found., Conclusions: Intraretinal crystals are a frequent phenomenon associated with type 2 MacTel. They may appear at all stages and aid in the early diagnosis of the disease. Their morphology further implicates Müller cells in the pathogenesis of the disease. Insight into their physical and chemical properties may provide clues to the metabolic pathways involved in the pathogenesis of the disease., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To determine the effect of treatment with intravitreal bevacizumab on retinal thickness and visual acuity in the nonproliferative and proliferative forms of Type 2 idiopathic macular telangiectasia., Methods: Retrospective chart review of clinic patients treated with bevacizumab for macular telangiectasia Type 2. Treatment was performed until no further changes were seen after repeated bevacizumab injections. All patients had Snellen visual acuity testing, fundus fluorescein angiography, and measurement of central macular thickness by optical coherence tomography at baseline. Visual acuity and central macular thickness were recorded at follow-up visits., Results: Fourteen eyes of 10 patients were included. In 5 eyes with nonproliferative macular telangiectasia Type 2, average follow-up was 17 months (± 7 months), and no eye demonstrated improvement in visual acuity or decrease in central macular thickness at final follow-up compared with baseline. In 9 eyes with proliferative disease, follow-up averaged 17 months (± 9 months). At 6 weeks, central macular thickness decreased 63 μm (± 58 μm), and acuity improved 1.7 lines (± 2 lines). At final follow-up, central macular thickness decreased 48 μm (± 89 μm) and acuity improved 1.1 lines (± 3 lines). Subretinal neovascularization resolved in eight of nine eyes with proliferative disease after treatment., Conclusion: Bevacizumab did not improve acuity or reduce retinal thickness in nonproliferative macular telangiectasia Type 2 at final follow-up. In proliferative macular telangiectasia Type 2, bevacizumab caused involution of neovascularization and improved visual acuity.

Purpose: To investigate the efficacy and safety of intravitreal ranibizumab for the treatment of nonproliferative macular telangiectasia (MacTel) type 2., Design: Prospective, open-label, uncontrolled, nonrandomized interventional clinical trial., Methods: One eye (disease stage 2 or 3) of each patient (n = 10) with nonproliferative MacTel type 2 was injected with 0.5 mg ranibizumab at monthly intervals for one year. Visual acuity, angiographic findings, and retinal thickness were assessed at each visit. The primary endpoint was the change in best-corrected distance visual acuity after one year compared to baseline., Results: Mean visual acuity showed a transient increase in the study eye. However, after 12 months of treatment there was no significant change of visual acuity compared to baseline or compared to the fellow eye. Fluorescein angiography revealed a decrease of telangiectatic-appearing capillaries and of late-phase leakage, which was accompanied by a topographically related significant reduction in macular thickness. Three to 5 months after the last treatment, angiographic appearance and retinal thickness were similar to baseline. In one patient, the last intravitreal injection was not performed because of safety concerns after a transitory ischemic attack. Otherwise, no serious adverse events were observed., Conclusions: The angiographic and tomographic effects after intravitreal inhibition of vascular endothelial growth factor (VEGF) using ranibizumab implicate a pathophysiological role of the VEGF pathway in nonproliferative MacTel type 2. As the morphologic response was not associated with a clear functional benefit, and because of the transient nature of the treatment effect, monthly intravitreal ranibizumab is not recommended for the nonproliferative disease stage of MacTel type 2., (Copyright © 2011 Elsevier Inc. All rights reserved.)