Idiopathic Juxtafoveal Retinal Telangiectasis Research Articles

Multicolor imaging in macular telangiectasia-a comparison with fundus autofluorescence.

To describe various clinical features of idiopathic juxtafoveal retinal telangiectasis group 2A or idiopathic macular telangiectasia type 2 (MacTel) on multicolor imaging (MCI) and compare imaging findings of MacTel on MCI with fundus autofluorescence (FAF). Patients with a clinical diagnosis of MacTel based on Gass and Blodi's classification were included. FAF and MCI images were graded qualitatively for stage of disease, margins of involvement, hyperautofluorescence on FAF (corresponding retinal atrophy on MCI), and detection of crystals. FAF and MCI were graded quantitatively for the area and number of quadrants involved, hypoautofluorescene on FAF (corresponding intraretinal pigment hyperplasia or retinal pigment epithelium [RPE] atrophy on MCI), and foci of right-angled venules. Seventy-eight eyes of forty five patients were included with both imaging modalities showing no difference with respect tostaging of non-proliferative MacTel. Retinal crystals were recognized on MCI but not on FAF. Neurosensory retinal atrophy and subretinal neovascular membranes were detected using MCI with 92.3 and 83.3% sensitivity, respectively. Intraretinal pigmented hyperplasia was more accurately detected (70.1 vs 58.4%) compared with RPE atrophy on MCI. MCI showed larger area of involvement, higher number of quadrants involved (p < 0.001), and better delineation of margins (p = 0.002) compared with FAF. A higher mean number of vessel dipping foci was noted on MCI in comparison with FAF (3.34 vs 3.1). Various parameters were more easily defined using MCI compared with FAF which qualifies MCI as an enface depth-resolved imaging adjunct to conventional multimodal imaging in MacTel. The ability to detect enface as well as cross-sectional imaging features makes MCI a valuable tool in MacTel.

Genome-wide analyses identify common variants associated with macular telangiectasia type 2.

Idiopathic juxtafoveal retinal telangiectasis type 2 (macular telangiectasia type 2; MacTel) is a rare neurovascular degenerative retinal disease. To identify genetic susceptibility loci for MacTel, we performed a genome-wide association study (GWAS) with 476 cases and 1,733 controls of European ancestry. Genome-wide significant associations (P < 5 × 10-8) were identified at three independent loci (rs73171800 at 5q14.3, P = 7.74 × 10-17; rs715 at 2q34, P = 9.97 × 10-14; rs477992 at 1p12, P = 2.60 × 10-12) and then replicated (P < 0.01) in an independent cohort of 172 cases and 1,134 controls. The 5q14.3 locus is known to associate with variation in retinal vascular diameter, and the 2q34 and 1p12 loci have been implicated in the glycine/serine metabolic pathway. We subsequently found significant differences in blood serum levels of glycine (P = 4.04 × 10-6) and serine (P = 2.48 × 10-4) between MacTel cases and controls.

Intravitreal bevacizumab in the treatment of idiopathic juxtafoveal telangiectasis type I

Clinical caseWe report a case of a 42-year-old male with a macular edema due to idiopathic juxtafoveal retinal telangiectasis type I, treated with 3 sequential injections of intravitreal bevacizumab (1.25mg in 0.05ml). Anatomical improvements were observed after one year of follow up. DiscussionThere is currently no general consensus regarding the treatment of unilateral idiopathic juxtafoveal telangiectasis. The therapeutic options are, grid laser photocoagulation, intravitreal triamcinolone, verteporfin photodynamic therapy, or anti-VEGF. Visual acuity and anatomical improvements were observed in this case after intravitreal bevacizumab. Thus, intravitreal bevacizumab seems to be effective to treat macular edema in idiopathic juxtafoveal telangiectasis type I.

Diagnosis of subretinal neovascularization associated with idiopathic juxtafoveal retinal telangiectasia — fluorescein angiography versus spectral-domain optical coherence tomography

To compare ability of fluorescein angiography (FFA) and spectral-domain optical coherence tomography (SD-OCT) to diagnose subretinal neovascularization (SRNV) secondary to idiopathic juxtafoveal retinal telangiectasia (IJRT) type 2A. Two masked observers evaluated FFA and SD-OCT images separately to diagnose the presence of SRNV in 65 eyes of 36 patients. A third masked observer diagnosed SRNV on color fundus photo. Presence of SRNV on color fundus photo was defined as presence of subretinal hemorrhage, thickening of retina, and/or visible membrane at the macula. Presence of SRNV on FFA was defined when there was hyperfluorescence in early phase with increase in intensity and size in the late phase; presence of membrane, large irregular lesion, hypofluorescence due to subretinal haemorrhage. SRNV on SD-OCT was defined as the presence of thickening of the retinal pigment epithelium-choriocapillaris complex with or without intraretinal fluid or subretinal fluid and associated with intraretinal hyperreflective area with retinal thickening. Interobserver agreement (Kappa) for diagnosis of SRNV on FFA and SD-OCT was 0.373 (95 % CI, 0.106-0.617) and 0.775 (95 % CI, 0.612-0.899) respectively. The sensitivity and specificity of FFA were 52.3 % and 70.0 % respectively. With regard to SD-OCT, the sensitivity and specificity were 72.7 % and 64.1 % respectively in reference to color photograph. The negative predictive value (NPV) of SD-OCT (80.6 %) was higher than FFA (73.7 %). When we considered only the presence of subretinal hemorrhage on color photograph as a confirmed diagnosis of SRNV and compared the diagnostic ability of FFA and SD-OCT, we found that the FFA had poor sensitivity (58.3 %) but a better specificity (71.8 %) than SD-OCT, which had sensitivity of 75 % and specificity of 64 % (Tables 1 and 2). However, the negative predictive value (NPV) of SD-OCT (89.29 %) was slightly better than FFA (84.85 %). Interobserver agreement between the observers was better for SD-OCT than for FFA in making the diagnosis of SRNV. SD-OCT is a better diagnostic modality than FFA for ruling out the presence of SRNV.

Bevacizumab intravítreo como tratamiento de las telangiectasias yuxtafoveales idiopáticas tipo i

Clinical caseWe report a case of a 42 year-old male with a macular edema due to idiopathic juxtafoveal retinal telangiectasis type i, treated with 3 sequential injections of intravitreal bevacizumab (1.25mg in 0.05ml). Anatomical improvements were observed after one year of follow up. DiscussionThere is currently no general consensus regarding the treatment of unilateral idiopathic juxtafoveal telangiectasis. The therapeutic options are, grid laser photocoagulation, intravitreal triamcinolone, verteporfin photodynamic therapy, or anti-VEGF. Visual acuity and anatomical improvements were observed in this case after intravitreal bevacizumab. Thus, intravitreal bevacizumab seems to be effective to treat macular edema in idiopathic juxtafoveal telangiectasis type i.

Indocyanine-green-mediated photothrombosis with intravitreal bevacizumab in idiopathic juxtafoveal retinal telangiectasis

Indocyanine-green-mediated photothrombosis with intravitreal bevacizumab in idiopathic juxtafoveal retinal telangiectasis

Spectral-domain optical coherence tomography findings in idiopathic juxtafoveal retinal telangiectasis type 2a

PURPOSE: To describe the spectral-domain optical coherence tomography (OCT) findings in a patient with idiopathic juxtafoveal retinal telangiectasis (IJRT) type 2A and correlate them with the fluorescein angiography (FA) signs. METHODS: Case report. PATIENT: A 59 years old male patient presented with slow decrease in vision and metamorphopsia, most prominent in his left eye. The ophthalmoscopic and FA examination set the diagnosis of IJRT type 2A nonproliferative stage 3 in the right eye and proliferative stage 5 in the left eye [2]. RESULTS: The spectral-domain OCT revealed thickness decrease and reflectivity attenuation of the outer layers, deep intraretinal vessels located in the outer nuclear layer in the right eye and presence of a subretinal neovascularisation (SRN), undisrupted retinal pigment epithelium (RPE) and disintegration of the outer layers in the left eye. There was not presence of cystoid spaces, internal limiting membrane draping or pigment hyperplasia lesions in both eyes. The extensive leakage on FA seemed not to correspond to the relatively normal in thickness retina in both eyes. Spectral-domain OCT showed the intraretinal location of the SRN and the integrity of the RPE over the entire macula that could facilitate the differentiation between neovascular IJRT and choroidal neovascularisation from various ethiology or retinal angiomatous proliferation. CONCLUSION: IJRT type 2A presents with variable OCT characteristics. Ophthalmoscopy and FA are the routine methods for disease diagnosis. Spectral-domain OCT could further distinguish changes in retinal microstructure. It could be a helpful tool in differentiating IJRT from other entities. The importance of OCT for early diagnosis, evaluation of prognostic factors and monitoring the follow-up could be elucidated in longitudinal prospective studies.

STRUCTURAL AND FUNCTIONAL CHANGES OVER TIME IN MacTel PATIENTS

To investigate functional and morphologic alterations over a 1-year review analysis in patients with type 2 idiopathic juxtafoveal retinal telangiectasia (MacTel). Nine eyes of 9 patients with MacTel underwent repeated scotopic and photopic fine matrix mapping (FMM), 10-2 photopic microperimetry, and imaging studies. Early Treatment Diabetic Retinopathy Study visual acuity assessment showed a median difference between examinations of 1.0 letter (range, -3 to 4 letters). The difference of sensitivity values of all test points was 4.5 dB (range, 0.4 -5.5 dB) for microperimetry 1, 0.4 dB (range, -0.8 to 1.7 dB) for photopic, and -1.7 dB (range, -6.1 to 1.0 dB) for scotopic fine matrix mapping, respectively. The difference in test points of more than a 10-dB loss compared with age-matched controls was higher for scotopic than for photopic testing (P= 0.03, Wilcoxon signed ranks test). Small progression of scotoma correlated with a slight increase in retinal blood vessel dilatation and hyperfluorescence and subtle enlargement of pigmented plaques. Changes in central visual acuity and microperimetry testing after 1 year most likely do not extend beyond test-retest variability. The deterioration of scotopic sensitivity confirms our previous results of more severe rod compared with cone dysfunction in MacTel. Changes in fine detail visual function over a 1-year period may be useful parameters for interventional trials.

Intravitreal ranibizumab as primary treatment for neovascular membrane associated with idiopathic juxtafoveal retinal telangiectasia

Intravitreal ranibizumab as primary treatment for neovascular membrane associated with idiopathic juxtafoveal retinal telangiectasia

A case of subretinal neovascularization treated with intravitreal ranibizumab in a patient with idiopathic juxtafoveal retinal telangiectasis

A 65-year-old lady presented with decreased vision in her left eye (LE). Best corrected visual acuity (BCVA) was 1/20. Complete examination showed idiopathic juxtafoveal retinal telangiectasis associated with subretinal neovascularization and she was treated with intravitreal ranibizumab every month for three months in the LE. After four months, her BCVA increased to 3/10. Fluorescein angiography (FA) showed minimal leakage and optical coherence tomography (OCT) confirmed absence of intra- or subretinal fluid in the macula. Examinations were repeated monthly for another 12 months and showed no recurrence. Intravitreal ranibizumab showed promising results for subretinal neovascularization due to idiopathic juxtafoveal retinal telangiectasis. A prospective study with large series of patients and controls may be necessary in order to determine the effectiveness of this treatment.

Idiopathic juxtafoveal retinal telangiectasia

Background Idiopathic juxtafoveal retinal telangiectasia (IJRT) is a rare retinal condition that can be detected even in asymptomatic patients. The most common appearance, type 2A, typically presents as juxtafoveolar telangiectasias with minimal exudation, superficial retinal crystalline deposits, and right-angle venules. Though initially asymptomatic, late complications such as foveolar atrophy and subretinal neovascularization can develop and should be recognized and treated accordingly. Case Report Two patients with IJRT are presented. The first patient, a 60-year-old Hispanic man, had asymptomatic type 2A IJRT with subretinal neovascularization diagnosed for which he underwent photodynamic therapy on 2 separate occasions. Currently, his visual acuity remains stable at 20/30 in the treated eye. The second patient, a 78-year-old man, had bilateral type 2A IJRT with decreased vision. Fluorescein angiography found no active leakage, and optical coherence tomography found significant foveal atrophy. Therefore, no treatment was advised, and the patient remains with acuities of 20/100- in the right eye and 20/80- in the left. Conclusion Initially, retinal signs can be subtle in type 2A IJRT, with no treatment being indicated. In the late stages of the disease, laser photocoagulation may be necessary to treat subretinal neovascular membranes, which can result as a complication, leading to dramatic vision loss if the fovea is affected. Careful inspection, including the use of fluorescein angiography and ocular coherence tomography, is often helpful in monitoring these patients.

Correlation of Functional Impairment and Morphological Alterations in Patients With Group 2A Idiopathic Juxtafoveal Retinal Telangiectasia

To correlate functional impairment with morphological alterations in patients with group 2A idiopathic juxtafoveal retinal telangiectasia. As part of the Macular Telangiectasia Project, a cohort of 10 patients underwent additional functional testing and imaging studies including photopic and scotopic fine matrix mapping, microperimetry, reflectance, and autofluorescence imaging with scanning laser ophthalmoscopy. From clinical stage 2 to 5, scotopic central function was reduced, which corresponded to depletion of macular pigment density. From clinical stage 3 onward, severe photopic and scotopic scotomata with up to 30 dB of loss were found next to fixation and were not totally confined to abnormalities seen with standard imaging modalities. The number of test points with loss of 10 dB or more was significantly greater for scotopic testing than for photopic testing (P = .007, Wilcoxon signed rank test). Rod function may be more severely affected than cone function in patients with group 2A idiopathic juxtafoveal retinal telangiectasia, and this may occur early in the disease progression. Severe reduction in retinal sensitivity is spatially confined to morphological alterations seen with scanning laser ophthalmoscopy imaging. The findings imply that idiopathic juxtafoveal retinal telangiectasia is not solely a vascular disease and that early neuronal involvement may be implicated in the pathogenesis of the disease.

Correspondence

Purpose: To determine the optical coherence tomography (OCT) findings in eyes with group 2a idiopathic juxtafoveal retinal telangiectasis (IJRT). Methods: Forty-one eyes of 22 patients with nonproliferative group 2a IJRT were examined. OCT testing including retinal topographic mapping and analysis, and horizontal and vertical line scans, was obtained on each eye. Results: None of the 41 eyes had a thickened foveal center. The average center foveal thickness was 166 μm (31–264 μm). Stage 1 eyes (n = 2) were normal fellow eyes in patients with contralateral group 2a IJRT. Stage 2 eyes (n = 11) all had parafoveolar temporal graying and intraretinal temporal fluorescein leakage, but rarely had photoreceptor disruption (18%) on OCT testing. Stage 3 eyes (n = 14) all had clinical and fluorescein findings similar to or more pronounced than stage 2 eyes. All stage 3 eyes also had one or more foveal cysts at various retinal depths on OCT. Most of these eyes (86%) had photoreceptor disruption and outer retinal atrophy on OCT. Stage 4 eyes (n = 14) all had a black foveal or parafoveolar pigment plaque and intraretinal temporal fluorescein leakage. All stage 4 eyes had a hyper-reflective plaque with shadowing on OCT corresponding to the pigment plaque. Most of these eyes had one or more foveal cysts (64%) and all of these eyes had photoreceptor disruption and outer retinal atrophy. Conclusion: OCT helps in the staging of group 2a IJRT and reveals multiple retinal structural abnormalities.

Macular edema associated with latanoprost use in a patient with idiopathic juxtafoveal retinal telangiectasis

Macular edema associated with latanoprost use in a patient with idiopathic juxtafoveal retinal telangiectasis

Intravitreal bevacizumab treatment of retinal telangiectasis complicated by subretinal neovascularization.

Idiopathic perifoveal telangiectasia (IPT) is a disorder characterized by focal parafoveal capillary dilatation of unknown cause. Photodynamic therapy (PDT) was reported to be efficacious in the treatment of subretinal neovascularization (SRN) associated with idiopathic juxtafoveal retinal telangiectasis. However, visual improvement is limited. We describe the clinical, angiographic, and optical coherence tomography findings for a patient with IPT complicated with SRN and treated with PDT and bevacizumab. The patient underwent 2 sessions of PDT; however, metamorphopsia persisted, and visual acuity further decreased to 20/100 after the second PDT. Two injections of intravitreal bevacizumab (1.25 mg) were administered after PDT. Visual acuity increased dramatically from 20/100 to 20/25 after the injections. Four months after the second injection, there was no recurrence, and the patient was asymptomatic. Intravitreal bevacizumab treatment should be considered for patients with SRN associated with perifoveal telangiectasis, especially after insufficient therapeutic response to PDT.

Intravitreal Bevacizumab for Macular Edema From Idiopathic Juxtafoveal Retinal Telangiectasis

To assess the potential visual benefit of intravitreal bevacizumab in a patient with idiopathic juxtafoveal retinal telangiectasis refractory to focal laser treatment, an intravitreal injection of bevacizumab (1.25 mg) was given. Within 1 week, visual acuity improved from 20/50 to 20/25 and optical coherence tomography demonstrated complete resolution of macular edema. There was no adverse effect. The macular edema recurred after 3 months, requiring a repeat injection of bevacizumab with subsequent resolution of macular edema. An intravitreal injection of bevacizumab may provide potential short-term visual benefit in patients with macular edema from idiopathic juxtafoveal retinal telangiectasis.

OPTICAL COHERENCE TOMOGRAPHY FINDINGS IN NONPROLIFERATIVE GROUP 2A IDIOPATHIC JUXTAFOVEAL RETINAL TELANGIECTASIS

To determine the optical coherence tomography (OCT) findings in eyes with group 2a idiopathic juxtafoveal retinal telangiectasis (IJRT). Forty-one eyes of 22 patients with nonproliferative group 2a IJRT were examined. OCT testing including retinal topographic mapping and analysis, and horizontal and vertical line scans, was obtained on each eye. None of the 41 eyes had a thickened foveal center. The average center foveal thickness was 166 microm (31-264 microm). Stage 1 eyes (n = 2) were normal fellow eyes in patients with contralateral group 2a IJRT. Stage 2 eyes (n = 11) all had parafoveolar temporal graying and intraretinal temporal fluorescein leakage, but rarely had photoreceptor disruption (18%) on OCT testing. Stage 3 eyes (n = 14) all had clinical and fluorescein findings similar to or more pronounced than stage 2 eyes. All stage 3 eyes also had one or more foveal cysts at various retinal depths on OCT. Most of these eyes (86%) had photoreceptor disruption and outer retinal atrophy on OCT. Stage 4 eyes (n = 14) all had a black foveal or parafoveolar pigment plaque and intraretinal temporal fluorescein leakage. All stage 4 eyes had a hyper-reflective plaque with shadowing on OCT corresponding to the pigment plaque. Most of these eyes had one or more foveal cysts (64%) and all of these eyes had photoreceptor disruption and outer retinal atrophy. OCT helps in the staging of group 2a IJRT and reveals multiple retinal structural abnormalities.

Optical coherence tomography findings in idiopathic juxtafoveal retinal telangiectasis

To describe optical coherence tomography (OCT) findings in patients with juxtafoveal retinal telangiectasis (JRT). Fourteen consecutive patients (28 eyes) with JRT (12 patients with JRT type II, one with JRT type I and one with JRT type III) were examined using fluorescein angiography (FA) and OCT. Despite prominent leakage in FA, macular oedema was absent in all 26 eyes with type II JRT. In contrast to that, in patients with type I and type III JRT, cystoid macular oedema was evident. In 14 of 28 eyes (all with type II JRT), a single foveal cyst was found in OCT. It varied significantly in size and was associated with visual acuity decrease. An intraretinal hyperreflective lesion was seen in eight of 28 eyes and flattening of the fovea in three eyes. Foveal cyst, absent macular oedema, intraretinal hyperreflective lesions and foveal flattening were the most common OCT findings in patients with JRT type II. These may represent progressive loss of retinal tissue, possibly due to Müller cells degeneration, and provide additional diagnostic criteria for JRT.

Idiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography

To investigate the capabilities of ultrahigh-resolution optical coherence tomography (UHR OCT); to compare with the commercially available OCT standard-resolution system, StratusOCT, for imaging of idiopathic juxtafoveal retinal telangiectasis (IJT); and to demonstrate that UHR OCT provides additional information on disease morphology, pathogenesis, and management. Retrospective, observational, interventional case series. Nineteen eyes of 10 patients diagnosed with IJT in at least one eye. All patients were imaged with UHR OCT and StratusOCT at the same visit. A subset of patients was also imaged before and after treatment of IJT. Ultrahigh- and standard-resolution cross-sectional tomograms of IJT pathology. Using both standard- and ultrahigh-resolution OCT, we identified the following features of IJT: (1) a lack of correlation between retinal thickening on OCT and leakage on fluorescein angiography, (2) loss and disruption of the photoreceptor layer, (3) cystlike structures in the foveola and within internal retinal layers such as the inner nuclear or ganglion cell layers, (4) a unique internal limiting membrane draping across the foveola related to an underlying loss of tissue, (5) intraretinal neovascularization near the fovea, and (6) central intraretinal deposits and plaques. In 63% of cases, the presence of abnormal vessels and a discontinuity of the photoreceptor layer correlated with visual acuity. Ultrahigh-resolution OCT improves visualization of the retinal pathology associated with IJT and allows identification of new features associated with it. Some of these features, such as discontinuity of the photoreceptor layer, are revealed only by UHR OCT.