Idiopathic Macular Telangiectasia Type Research Articles

FROM OUTER RETINAL NEOVACULARIZATION TO EXUDATIVE SUBRETINAL NEOVASCULARIZATION IN MACULAR TELANGIECTASIA TYPE 2

Purpose: To describe the progression from outer retinal neovascularization (ORNV) to exudative subretinal new vessels (SRNV) in idiopathic macular telangiectasia type 2 (MacTel2) Patients and Methods: A total of 135 patients (270 eyes) imaged with optical coherence tomography angiography (OCTA) were included. Main Outcome Measures: Ellipsoid zone (EZ) loss, outer retinal hyperreflectivity (ORHR), ORNV, and SRNV. ORNV was defined as a flow signal passing through the outer plexiform layer, with or without vertical linear ORHR on the OCT B-scan. SRNV were defined as an abnormal capillary network with a peripheral anastomotic arcade seen on en face OCTA, and a convex hyperreflectivity at the retinal pigment epithelium. Results: SRNV were observed in 38/270 eyes (14%). SRNV were at a fibrotic stage in 24/38 eyes, at an exudative stage in 6/38 eyes, and a progression from ORNV to SRNV was documented in 8/38 eyes. All cases showed an EZ loss. In 7 eyes (2.5%), SRNV were also associated with subepithelial neovascularization. No retinochoroidal anastomosis was detected. The visual acuity dropped when SRNV were present. Conclusion: In this case series, SRNV were found in 14% of eyes. In all cases, they were associated with an EZ loss and with ORHR. A progression from ORNV to SRNV was observed.

Proximal nailfold videocapillaroscopy findings of patients with idiopathic macular telangiectasia type 2.

Idiopathic macular telangiectasia type 2 (IMT) is a neurodegenerative disease characterized by bilateral, idiopathic, and perifoveal retinal telangiectatic vessel formations. We aimed to compare proximal nailfold videocapillaroscopy (NV) findings between patients with IMT and healthy individuals and evaluate the optical coherence tomography angiography (OCTA) parameters of the patients with IMT according to their NV findings. The study included 43 patients with IMT and 92 healthy controls of similar age and gender without any additional diseases. The OCTA and NV findings of the patients and controls were examined. The mean age was 59.76 ± 5.73 years in the IMT group and 58.23 ± 4.96 years in the control group. Of the 43 patients with IMT, 19 were found to have increased capillary tortuosity, six had microhemorrhage, and 18 had bizarre capillaries (P < 0.001). In the IMT group, the total vascular density value of the superficial capillary plexus was higher among the patients with capillary microhemorrhage (P = 0.001), and the subfoveal choroidal thickness was lower among those with increased capillary tortuosity and bizarre capillaries (P = 0.04 and P = 0.07, respectively). This is the first study in which the NV findings of patients with IMT were compared with those of a control group. We found higher rates of increased capillary tortuosity, microhemorrhage, and bizarre capillaries in the IMT group compared to the controls. We consider that this situation is caused by microvascular damage. We also think that IMT is a systemic disease that affects both proximal nailfold capillaries and eye vessels.

Evaluation of Optic Disc, Retinal Vascular Structures, and Acircularity Index in Patients with Idiopathic Macular Telangiectasia Type 2.

We aimed to compare the retinal, optic disc vascular density (ODVD) values, and acircularity index (AI) of patients with idiopathic macular telangiectasia type 2 (IMT) and healthy individuals using the optical coherence tomography angiography (OCTA) device. The study included 39 patients with IMT and 37 healthy controls. The OCTA findings of the patients and controls were examined. The total, parafoveal and perifoveal vascular density of the superficial capillary plexus, choriocapillaris blood flow, inside-disc ODVD, retinal nerve fiber layer (RNFL), and retinal thicknesses were found to be statistically significantly lower, and the foveal avascular zone value was statistically significantly higher in the IMT group compared to the control group (p = 0.001, p = 0.01, p = 0.02, p = 0.01, p = 0.009, p = 0.002, p = 0.02, respectively). There was a statistically significant negative correlation between best-corrected visual acuity (BCVA) and AI (p = 0.02), and a statistically significant positive correlation between peripapillary vascular density and BCVA (p = 0.04). We consider that the lower retinal, choriocapillaris, ODVD values, and retinal and RNFL thicknesses in the patients with IMT compared to the controls were due to vascular damage, remodeling, fibrosis, proliferation, and Müller cell damage. Ellipsoid zone defect, AI, and peripapillary vascular density are important indicators in the evaluation of visual acuity in these patients.

Idiopathic Macular Telangiectasia

Idiopathic macular telangiectasia is a disease characterized by retinal capillary ectasia in the parafoveal temporal region of the macula. The disease is divided into two main groups: aneurysmal telangiectasia or idiopathic macular telangiectasia type 1, which include congenital, unilateral, and exudative juxtafoveal telangiectasia, occurring most commonly in males and cause visual impairment due to exudation and macular edema; and perifoveal telangiectasia or idiopathic macular telangiectasia type 2, which is the more common type of the disease, occurring in middle-aged men and women and bilateral.

Idiopathic macular telangiectasia type 2, stage V or proliferative stage: Multimodal imaging

To compare the expression profiles of various cytokines and chemokines in vitreous samples from patients with retinal detachment (RD) to those from controls and to analyze their association with various clinical features.In this prospective study, undiluted vitreous fluid was obtained from 41 patients with primary RD and 33 controls with macular hole or vitreomacular traction. A multiplex bead immunoassay was performed to determine the expression of 27 inflammatory mediators.Eleven mediators were significantly upregulated in the vitreous of RD patients compared with controls, including the following: cytokines IL-1ra, IL-6, IL-7, IL-8, IFN-γ; chemokines CCL2, CCL3, CCL4, CXCL10 and CCL11 and growth factor G-CSF. Correlation analyses showed that levels of IL-1ra, CXCL10, CCL11 and G-CSF were positively correlated to the extent of detachment, while those of IL-1ra and CXCL10 were associated with the duration of detachment. There was also a positive association between the concentrations of CXCL10 and CCL11 and preoperative flare values. Additional analysis revealed that flare values and both CXCL10 and CCL11 levels were significantly higher in eyes with grade B or C proliferative vitreoretinopathy (PVR).Our results confirm that RD induces a marked inflammatory response with a complex cytokine network. We identified proteins specifically linked to several clinical features that might contribute to photoreceptor degeneration and PVR-related redetachment. These proteins may represent potential therapeutic targets for improving the anatomical and functional outcomes of RD surgery.Évaluer le profil des médiateurs d’inflammation dans le vitré des patients présentant un décollement de rétine (DR) rhegmatogène et corréler leur expression avec différents paramètres cliniques.Etude prospective portant sur 74 patients ayant bénéficié d’un prélèvement vitréen lors d’une vitrectomie pour un DR primaire (n = 41) ou un trou maculaire idiopathique (n = 33, témoins). Les échantillons de vitré ont été analysés selon une technique Luminex® visant à quantifier l’expression de 27 médiateurs inflammatoires.Onze médiateurs était significativement surexprimés dans le vitré des patients opérés de DR : les cytokines IL-1ra, IL-6, IL-7, IL-8, IFN-γ; les chimiokines CCL2, CCL3, CCL4, CXCL10 et CCL11 et le facteur de croissance G-CSF. Les analyses de corrélation ont montré que les taux d’IL-1ra, CXCL10, CCL11 et G-CSF étaient corrélés à l’étendue du DR tandis que ceux d’ IL-1ra et CXCL10 étaient associés à la durée du DR. Il existait également une forte association entre les concentrations de CXCL10 et CCL11 et le flare de l’humeur aqueuse en préopératoire. Les valeurs de flare et les taux de CXCL10 et CCL11 étaient par ailleurs significativement plus élevés dans les yeux avec prolifération vitréo-rétinienne (PVR) de stade B ou C.Nos résultats confirment que le DR induit une réponse inflammatoire sévère impliquant un réseau complexe de cytokines. Nous avons identifié plusieurs protéines spécifiquement associées à différents paramètres cliniques, susceptibles de contribuer à la dégénérescence des photorécepteurs et au développement de la PVR. Elles pourraient représenter des cibles thérapeutiques potentielles pour améliorer les résultats anatomiques et fonctionnels de la chirurgie du DR.

Internal limiting membrane graft in full-thickness macular hole secondary to macular telangiectasia type 2

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

Focal laser for idiopathic macular telangectasia type 1 refractory to ozurdex, ranibizumab and aflibercept

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

Outcome of Off-Label AREDS 2 Supplementation for the Treatment of Macular Degeneration in Non-Proliferative Idiopathic Type 2 Macular Telangiectasia.

PurposeTo evaluate if off-label Age-Related Eye Disease Study 2 (AREDS2) supplementation prevents visual and anatomical deterioration in non-proliferative Idiopathic Macular Telangiectasia Type 2 (IMT2).Patients and MethodsThis is a single-center retrospective, comparative study of 82 IMT2 eyes treated with AREDS2 from January 1st, 2013 to January 1st, 2018. The study analysis consisted of a non-comparative arm, which included all AREDS2 eyes, and a comparative arm (27 AREDS2 and 42 untreated eyes) that only included eyes with complete follow-up data. Eyes were evaluated at baseline, 12 and 24 months. Better/worse eye sub-analysis was performed in the comparative study arm. Primary outcomes were best corrected visual acuity (BCVA) and optical coherence tomography (OCT) anatomical characteristics including largest cavitation diameter, central macular thickness (CMT), and length of ellipsoid zone (EZ) loss at 24 months.ResultsIn the non-comparative arm, AREDS2 eyes showed stable BCVA (0.28 ± 0.18 logMAR at baseline vs 0.26 ± 0.19 logMAR at 24 months; p = 0.35) and OCT anatomical features after 24 months of supplementation. In the comparative arm, BCVA mean difference was greater for untreated eyes at 24 months (−0.09 ± 0.15 vs 0.03 ± 0.11 logMAR; p = <0.001). AREDS2 eyes had decreased cavitary diameter and EZ loss compared to untreated eyes at the study endpoint (p = 0.01 and p = 0.02, respectively). CMT remained stable for both cohorts throughout the study. For better/worse eye analysis, untreated eyes had worse BCVA at 24 months in both better and worse eyes (both p = 0.01). For anatomical outcomes, increases in both EZ loss (p = 0.04) and cavitary diameter (p = 0.001) among untreated eyes were only significant for eyes with worse baseline BCVA.ConclusionOur results suggest that off-label AREDS2 supplementation in non-proliferative IMT2 may prevent anatomical and visual deterioration in a subset of eyes.

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.

Optical Coherence Tomography Angiography Features of Idiopathic Macular Telangiectasia Type 2 and Macular Branch Retinal Vein Occlusion

Optical Coherence Tomography Angiography Features of Idiopathic Macular Telangiectasia Type 2 and Macular Branch Retinal Vein Occlusion

Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2

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

Inverted internal limiting membrane-flap technique to treat a full-thickness macular hole complicating idiopathic macular telangiectasia type 2

Full-thickness macular hole is a complication of idiopathic macular telangiectasia type 2 with a low surgical closure rate and a poor functional recovery. We analyze morphologic and functional outcomes after an inverted internal limiting membrane-flap technique for full-thickness macular hole complicating idiopathic macular telangiectasia type 2 using optical coherence tomography and microperimetry. A 77-year-old man presented us complaining visual impairment in the left eye (20/40) due to a full-thickness macular hole complicating idiopathic macular telangiectasia type 2. The patient underwent vitrectomy with an inverted internal limiting membrane-flap technique and injection of 22% SF6 gas. Optical coherence tomography and functional analysis, including visual acuity test and microperimetry, were performed before surgery and over 3 months. Optical coherence tomography scans revealed macular hole closure at 1-month follow-up. Visual acuity and retinal sensitivity improved from 20/40 to 20/20 and from 13.1 to 14.9 dB, respectively. Fixation stability (bivariate contour ellipse area) improved from 2.02 to 1.58 deg2, from 5.44 to 4.24 deg2 and from 9.72 to 7.58 deg2, at 68%, 95%, and 99% of fixation points, respectively. Inverted internal limiting membrane-flap technique may be an alternative approach in patients with full-thickness macular hole complicating diopathic macular telangiectasia type 2, and microperimetry may be a useful tool to analyze related functional changes.

Management of Idiopathic Macular Telangiectasia Type 2.

Macular telangiectasia type 2 (MacTel) is a relatively rare disease without established treatments. Although MacTel was previously considered a primarily vascular condition, the thinking on its pathogenesis has shifted to it now being considered principally a neurodegenerative disease. This has resulted in a subsequent change in the approach to treatment toward neuro-protection for the non-proliferative phase of this disease. Carotenoid supplementation has had mixed results. Ciliary neurotrophic factor (CNTF) has demonstrated some promising early results, but further study is necessary to determine its actual effect. Some structural improvements have been seen in the non-proliferative phase with oral acetazolamide but without accompanying functional improvement. Anti-vascular endothelial drugs have been studied and not found to have benefit in the non-proliferative phase of disease but have demonstrated significant structural and functional value in the treatment of secondary neovascularization. There is no level I evidence for the various proposed MacTel treatments, and efforts need to be directed toward conducting multicenter randomized trials to better understand possible treatments for this condition.Electronic supplementary materialThe online version of this article (10.1007/s40123-019-0170-1) contains supplementary material, which is available to authorized users.

Idiopathic Macular Telangiectasia Type 2 and Its Relationship with Macular Pigment Optical Density

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

No association between sleep apnoea and macular telangiectasia type 2 and its markers of severity and progression: a case-control study and retrospective cohort study.

Idiopathic Macular Telangiectasia Type 2 (MacTel) is an uncommon, progressive retinal disease usually affecting both eyes. Currently there is no know treatment however with similar comorbidities to Obstructive Sleep Apnoea (OSA) there is plausibility of an association which may accelerate disease progression. To identify an association between MacTel and OSA and whether OSA may result in increased disease progression. Matched case-control study and retrospective cohort analysis. Fifty-seven patients with MacTel and 165 matched controls from the Busselton Health Study. MacTel participants were matched based on age, gender and body mass index (BMI) (and where possible hypertension and diabetes) on a 3:1 ratio with controls from the Busselton Health Study. Participants undertook a sleep questionnaire using a previously validated questionnaire. In a subset sleep apnoea severity was objectively measured via overnight ambulatory polygraphy (30 cases and 83 matched controls; ApneaLink device; ResMed, Sydney, Australia). In a retrospective analysis of the suspected MacTel cases we assessed whether major markers of OSA severity and MacTel progression were associated. Apnoea Hypopnea Index along with key markers of MacTel progression. MacTel patients did not have a higher risk of sleep apnoea when compared to age, sex and BMI -matched controls (mean ± SD Apnoea hypopnea index [AHI] cases 9.6 ± 14.7 vs. controls 9.7 ± 10.8, P = 0.95). No markers of disease progression in the cases were associated with any marker of OSA severity. Sleep apnoea does not increase the risk or accelerate the progression of MacTel.

Predictive multi-imaging biomarkers relevant for visual acuity in idiopathic macular telangiectasis type 1

BackgroundTo evaluate the structural changes associated with visual acuity (VA) in patients with idiopathic macular telangiectasia (MT) type 1 using multimodal imaging modalities.MethodsA retrospective study of 14 patients with MT type 1 and of 10 eyes from 10 healthy individuals as age-matched controls was conducted. The medical records of patients who had undergone colour fundus photography, spectral domain optical coherence tomography (OCT), fluorescein angiography and OCT angiography were reviewed. Central macular thickness (CMT), the areas of macular oedema and ellipsoid zone (EZ) disruption, EZ length, disorganization of the retinal inner layers (DRIL) and external limiting membrane (ELM) disruption, as measured by spectral domain OCT; and vascular density and the foveal avascular zones (FAZ) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP), as measured by OCT angiography, were assessed in MT type 1 eyes and correlated with VA.ResultsThe mean baseline best-corrected VA of MT type 1 eyes was 0.45 ± 0.28. The mean CMT was 385.19 ± 75.21 μm in MT type 1 eyes and 252.43 ± 15.77 μm in contralateral eyes (Z = − 4.113, p < 0.001). The mean vessel density of the DCP was lower in MT type 1 eyes (47.25 ± 4.69%) than in contralateral eyes (53.93 ± 2.94%) and normal eyes (59.37 ± 2.50%) (Z = − 3.492, − 4.099; p < 0.001, < 0.001). The baseline logMAR VA was correlated with CMT (r = 0.682, p = 0.007), SCP density (r = − 0.652, p = 0.012), DCP density (r = − 0.700, p = 0.005), total area of EZ disruption (r = 0.649, p = 0.012); and total lengths of EZ (r = 0.681, p = 0.007), ELM (r = 0.699, p = 0.005) and DRIL (r = 0.707, p = 0.005) disruption in the 1-mm-diameter foveal region in MT type 1 eyes.ConclusionsDecreased DCP density and the presence of DRIL may be predictive biomarkers of VA in MT type 1. CMT, SCP density, total area of EZ disruption, and lengths of EZ and ELM disruption within the 1-mm-diameter central region were strongly associated with VA.

Idiopathic macular telangiectasia type 2: Prevalence and a morphometric and phenotypic study

Type 2 idiopathic macular telangiectasia (MacTel 2) 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. A retrospective study was conducted on the 12 eyes of 6 patients with MacTel 2. Fundus color photographs, fundus autofluorescence, fluorescein angiography, optical coherence tomography (OCT), and OCT-angiography were performed and subsequently analyzed. Visual acuity (VA) was prospectively recorded. The prevalence was established based on the patients referred to a specialized macular diseases unit. Minimum follow-up period was 18 months. 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. 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. Las telangiectasias maculares idiopáticas de tipo 2 (MacTel 2) son una enfermedad retiniana poco frecuente y aún no completamente caracterizada. El objetivo del presente trabajo es describir las características clínicas en imagen multimodal de la retina, presentar los resultados funcionales y estimar la prevalencia de la enfermedad en nuestro medio. Estudio retrospectivo en el que se analizaron 12 ojos de 6 pacientes con MacTel 2. Se realizaron fotografías en color de fondo, autofluorescencia, angiografía fluoresceínica, tomografía de coherencia óptica (OCT) y angiografía por OCT y se analizaron posteriormente. Se registró prospectivamente la agudeza visual. La prevalencia se estableció en base en los pacientes referidos a una unidad especializada en enfermedades maculares. El período mínimo de seguimiento fue de 18 meses. La prevalencia de MacTel 2 en nuestro medio fue de 0,12%. Se presentaron las características clínicas en imagen multimodal de la retina. La agudeza visual permaneció estable durante el seguimiento (p = 0,492). Tres pacientes desarrollaron neovascularización coroidea (NVC), requiriendo tratamiento intravítreo con agentes antiangiogénicos. La prevalencia de la enfermedad en nuestro medio es del 0,12%. El estudio mediante imágenes multimodales permite un diagnóstico y seguimiento de esta patología más preciso. La agudeza visual se mantuvo durante los 18 meses de seguimiento. Los pacientes que desarrollan NVC y son tratados con agentes antiangiogénicos parecen responder adecuadamente a ellos. Se necesitan más estudios para establecer estas conclusiones.

CONCURRENT IDIOPATHIC MACULAR TELANGIECTASIA TYPE 2 AND CENTRAL SEROUS CHORIORETINOPATHY.

To describe cases presenting with features of idiopathic macular telangiectasia (MacTel) Type 2 and central serous chorioretinopathy (CSC). Databases from four tertiary retina centers were searched for cases copresenting CSC and MacTel Type 2. Five cases were identified (4 men, 1 woman; mean age: 67.2 years). Four patients were referred for chronic or nonresolving CSC, and the diagnosis of MacTel Type 2 was made based on multimodal imaging findings. One patient had advanced MacTel Type 2, and developed acute CSC. Regarding the MacTel Type 2 findings, all subjects presented perifoveal telangiectasia on fluorescein angiography, and four subjects showed intraretinal cavitations typical of MacTel Type 2 on optical coherence tomography, in one or both eyes. Regarding the CSC findings, fluorescein angiography identified focal or extended retinal pigment epithelium alteration in all eyes, and an active leakage in two eyes. Indocyanine green angiography showed choroidal vascular hyperpermeability in four subjects. On optical coherence tomography, pigment epithelial detachments were detected in five eyes (four subjects), and foveal detachments were present in five eyes (three subjects), which spontaneously resolved (two eyes), responded to photodynamic therapy (two eyes), or persisted (one eye). Mean choroidal thickness was 402 ± 99 μm. The codiagnosis of CSC and MacTel Type 2 should be considered in atypical presentations associating features from both disorders.

Global Connections to Study Idiopathic Macular Telangiectasia Type 2.

Global Connections to Study Idiopathic Macular Telangiectasia Type 2.

Diagnosis and management of a patient with idiopathic macular telangiectasia type 2

This is a case report discussing the case of a patient with idiopathic macular telangiectasia type 2. The diagnosis and the management of the patient are analysed as well as the possible factors that attribute to the appearance of idiopathic macular telangiectasia.