Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study-Report No. 1.

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.

Visual acuity outcomes in Coats disease by classification stage in 160 patients.

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.

Quantification of Retinal and Choriocapillaris Perfusion in Different Stages of Macular Telangiectasia Type 2.

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: An overview of classification, management and outcomes.

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.

Long-Term Outcomes of Total Exudative Retinal Detachments in Stage 3B Coats Disease.

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.

Idiopathic macular telangiectasia type 2: Prevalence and a morphometric and phenotypic study. / Telangiectasias maculares idiopáticas de tipo 2: prevalencia en nuestra área y estudio morfométrico y fenotípico.

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.

VISUAL FUNCTION CORRELATES OF FOVEAL SLOPE CHANGES ON OPTICAL COHERENCE TOMOGRAPHY IN MACULAR TELANGIECTASIA TYPE 2.

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.

SUBFOVEAL NODULE IN COATS' DISEASE: Toward an Updated Classification Predicting Visual Prognosis.

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).

Risk of Tractional Retinal Detachment Following Intravitreal Bevacizumab Along with Subretinal Fluid Drainage and Cryotherapy for Stage 3B Coats' Disease.

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.

Optical Coherence Tomography Angiography of Macular Telangiectasia Type 2.

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.

577-NM YELLOW LASER PHOTOCOAGULATION FOR COATS DISEASE.

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.

VERY EARLY DISEASE MANIFESTATIONS OF MACULAR TELANGIECTASIA TYPE 2.

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.

CONE DENSITY LOSS ON ADAPTIVE OPTICS IN EARLY MACULAR TELANGIECTASIA TYPE 2.

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.

Progression of Vision Loss in Macular Telangiectasia Type 2.

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.

Intraoperative fluorescein angiography-guided treatment in children with early Coats' disease.

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.

Treatment of stage 3 Coats' disease by endolaser photocoagulation via a two-port pars plana nonvitrectomy approach.

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.

Spectral-domain optical coherence tomography measurements of choroidal thickness and outer retinal disruption in macular telangiectasia type 2.

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.

A novel clinical sign in macular telangiectasia type 2.

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.

"En face" optical coherence tomography imaging in type 2 idiopathic macular telangiectasia.

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.