Original article
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
R.M. Bayón-Porrasa, I. Pascual-Campsb,
, C. Plaza-Laguardiac, R. Gallego-Pinazod
Corresponding author
a Servicio de Oftalmología, Hospital Nuestra Señora de la Montaña, Cáceres, Spain
b Servicio de Oftalmología, Hospital Universitario y Politécnico La Fe, Valencia, Spain
c Servicio de Oftalmología, Complejo Asistencial Universitario de León, León, Spain
d Unidad de Mácula, Clínica Oftalvist, Valencia, Spain
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In the second place, MacTel type 2 (MacTel 2) or perifoveal usually presents bilaterally and asymmetrically without visible aneurysms in conventional ocular fundus examination. In turn, MacTel 2 can be classified on the basis of the presence of secondary choroidal neovascularization (CNV) as nonproliferative—in the absence of this complication—and proliferative—when the presence of CMV is evidenced. Overall prevalence has been estimated at 0.1%.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The advent of new ocular fundus imaging diagnostic techniques has enabled greater knowledge of the physiopathological and structural substrate of MacTel 2 due to multiple clinic signs described with the analysis of various imaging forms. The objective of this manuscript is to summarize the characteristics of said pathology through multimode image and estimate the prevalence of the disease in the authors’ environment.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Material and methods</span><p id="par0015" class="elsevierStylePara elsevierViewall">The present study was designed as a retrospective case series. It included all patients with a clinical diagnostic of MacTel 2 visited by the same physician (RGP), a specialist in optical coherence tomography (OCT) interpretation at the Macula Unit of the University and Polytechnic Hospital of La Fe (Valencia, Spain). The study was carried out in accordance with the Helsinki declaration, the approval of the institutional board and the regulation of informed consent. The clinic and epidemiological characteristics of all patients were registered and analyzed together with the ocular fundus multimode image examination. The minimum follow-up during which comparisons were made was 18 months.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Best corrected visual acuity data were obtained with Snellen optotypes in decimal scale from the electronic clinic records of the patients. Similarly, the images obtained during the follow-up of each MacTel 2 patient were assessed. These included conventional color retinography (Visupac, Carl Zeiss Meditec, Jena, Germany), high resolution macular OCT images (HRA-OCT Spectralis, Heidelberg Engineering, Heidelberg, Germany), sodium fluorescein angiography (HRA-OCT Spectralis, Heidelberg Engineering, Heidelberg, Germany), ocular fundus shortwave autofluorescence (Blue Peak Spectralis, Heidelberg Engineering, Heidelberg, Germany), infrared reflectance (HRA-OCT Spectralis, Heidelberg Engineering, Heidelberg, Germany) and OCT angiography (OCT-A) by means of full-spectrum protocol (full-spectrum amplitude decorrelation angiography [FSADA]) (HRA-OCT Spectralis, Heidelberg Engineering, Heidelberg, Germany).</p><p id="par0025" class="elsevierStylePara elsevierViewall">The statistical study was made with nonparametric tests, specifically the Wilcoxon test for comparing mean values of related samples.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Results</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Epidemiological characteristics</span><p id="par0030" class="elsevierStylePara elsevierViewall">Overall, the study included 12 eyes of 6 patients with a clinic diagnostic of MacTel 2. In accordance with the registry comprising all 4903 patients visited by the same specialist (RGP) of the Macula Unit of the University and Polytechnic Hospital of La Fe, Valencia, Spain, between January 2013 and May 2017, the estimated prevalence being 0.12%.</p><p id="par0035" class="elsevierStylePara elsevierViewall">All patients were female, with a mean age of 63.00<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>6.03<span class="elsevierStyleHsp" style=""></span>years (range: 56–74; median: 63). The mean follow-up of all these cases was 5.17<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>3.72<span class="elsevierStyleHsp" style=""></span>years (range: 1–12; median: 4.5). As all patients had at least 18 months follow-up, visual acuity changes were registered randomly and assessed on the basis of said follow-up period.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Systemic associations in the sample of patients with MacTel 2 include the fact that 5 out of 6 patients (83%) exhibited systemic arterial hypertension during treatment, while 3 had diabetes mellitus type 2 (50%), and one was an active smoker (16%).</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Functional characteristics</span><p id="par0045" class="elsevierStylePara elsevierViewall">Best corrected visual acuity at diagnostic was 0.71<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>0.25 (range: 0.3–1.0; median: 0.7). Visual acuity at the end of the 18-month follow-up, was 0.71<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>0.26 (range: 0.3–1.0; median: 0.7). No statistically significant differences were observed (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.492).</p><p id="par0050" class="elsevierStylePara elsevierViewall">Mean intraocular pressure at diagnostic was 17.16<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>2.41<span class="elsevierStyleHsp" style=""></span>mmHg (range: 12–21; median: 17.5) and at the end, 18.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>2.69<span class="elsevierStyleHsp" style=""></span>mmHg (range: 15–25; median: 18). Statistically significant differences were observed in these values (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.010); however, none of the patients required antihypertensive treatment.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Albeit subjectively, all patients referred progressive loss of vision, particularly for reading, as well as progressive alteration in contrast sensitivity and adaptation from light to dark.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Phenotype characteristics in color retinography</span><p id="par0060" class="elsevierStylePara elsevierViewall">The main and most typical characteristic observed in all the retinographies of the eyes included in the study was the presence of grayish discoloration with loss of patency in the temporal juxtafoveal region (100%). Likewise, venule dilatation with right angle arrangement in the temporal juxtafoveal region was objectively observed in 12 eyes (100%). Finally, hyper-pigmented plates corresponding to retina pigment epithelium hyperplasia were observed in 3 eyes (25%), as well as crystalline deposits in 6 eyes (50%) (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Morphometric tomographic characteristics</span><p id="par0065" class="elsevierStylePara elsevierViewall">OCT images were characterized by the presence of hypo-reflective spaces that did not produce retinal thickness increases in all cases (100%), together with external retina layer disruption in 3 of the studied eyes (25%). <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a> comprises macular OCT images showing typical lesions as well as a case with CNV.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0070" class="elsevierStylePara elsevierViewall">Baseline foveal thickness was 237.7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>36.6<span class="elsevierStyleHsp" style=""></span>μm (range: 196–301<span class="elsevierStyleHsp" style=""></span>μm; median: 240<span class="elsevierStyleHsp" style=""></span>μm); at 18 months follow-up the thickness was 246.3<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>37.5<span class="elsevierStyleHsp" style=""></span>μm (range: 186–306<span class="elsevierStyleHsp" style=""></span>μm; median: 232<span class="elsevierStyleHsp" style=""></span>μm). No significant changes were observed (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.844).</p><p id="par0075" class="elsevierStylePara elsevierViewall">OCT analysis showed the development of CNV in 3 eyes (2 patients) that required intravitreal treatment with antiangiogenics medicaments (one injection in 2 eyes and 4 and the remainder) with remission of activity and subsequent visual acuity improvement up to previous levels. In accordance with the anatomic classification of neovascular lesions, this type of CNV was type 2 lesions in all cases.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Phenotype characteristics in ocular fundus autofluorescence</span><p id="par0080" class="elsevierStylePara elsevierViewall">Autofluorescence images of patients with this pathology initially showed loss of hypoautofluorescence, that was observed in all patients (100%). In one case, epithelium hyperplasia was also observed (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Phenotype characteristics in infrared reflectance</span><p id="par0085" class="elsevierStylePara elsevierViewall">Infrared reflectance images show a hypo-reflective perimacular ring and hyper-hypo-reflectance areas matching pseudocysts and retina pigment epithelium (RPE) hyperplasia, in addition to small reflectile crystals (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>).</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Phenotypic characteristics in fluorescein angiography</span><p id="par0090" class="elsevierStylePara elsevierViewall">Early stages of fluorescein angiography revealed little or no capillary dilatation evidence. Late stages showed perifoveal temporal retina staining respecting the central foveal area. With the progression of the disease, alterations completely surrounded the fovea. In the patients of this study, leak areas were observed above all in the temporal parafoveal area. In some cases the presence of CNV was also found in angiographic images (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>).</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Phenotypic characteristics in optic coherence tomography angiography</span><p id="par0095" class="elsevierStylePara elsevierViewall">OCT-A provides detailed images of retinal microcirculation. In the case of this pathology, vessel patency alterations are observed such as ectasic vessels with diffused or sectorial shape, distorted or even with neovascularization. The most frequent finding is vascular dilatation visible in the deep capillary plexus, which can progress and compromise the superficial capillary plexus. In addition, intravascular spaces increase with progressive capillary rarefaction and abnormal capillary anastomosis (<a class="elsevierStyleCrossRef" href="#fig0030">Fig. 6</a>).</p><elsevierMultimedia ident="fig0030"></elsevierMultimedia></span></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Discussion</span><p id="par0100" class="elsevierStylePara elsevierViewall">The prevalence of type 2 macular telangiectasia was established at approximately 0.1%.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">5</span></a> In the authors’ environment, a prevalence of 0.12% was found, similar to previously reported data.</p><p id="par0105" class="elsevierStylePara elsevierViewall">Type 2 macular telangiectasia do not exhibit gender predisposition. However, in the present case a predominance in females was observed as in other studies such as by Kim et al.<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">4</span></a> and Mehta et al.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">6</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">In a number of studies, a significant relationship has been observed between MacTel 2 and medical factors such as smoking, diabetes, arterial hypertension and obesity.<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">7</span></a> In the present study, the percentage of patients with arterial hypertension was 83%, of which 50% had diabetes and 16% were smokers, without finding other relevant pathological antecedents.</p><p id="par0115" class="elsevierStylePara elsevierViewall">Initially, MacTel 2 could be asymptomatic.<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">4</span></a> Several authors describe that visual acuity diminishes slowly in nonproliferative stages,<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">8</span></a> as observed by the authors in their patients. In contrast, the eventual appearance of CNV produces hemorrhages, fibrosis and neurosensory detachment with rapid and severe central vision impairment,<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">9</span></a> which could be recovered after treatment with antiangiogenics drugs.</p><p id="par0120" class="elsevierStylePara elsevierViewall">Various treatments have been applied for each stage of said pathology, including laser photocoagulation, photodynamic therapy, photothrombosis measured with indocyanine green, trans-pupil thermotherapy, surgery and medicaments such as acetazolamide, corticoids or intravitreal antiangiogenics as well as combined treatments.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a> Recent studies have suggested that laser treatment is not indicated due to risk of worsening and NVC.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a> Intravitreal antiangiogenic therapy has no effect on the evolution of the disease in nonproliferative cases, although it is the treatment of choice in neovascularization cases as it has demonstrated to improve visual acuity, resolve the CMV and diminish macular thickness.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a> There is not sufficient evidence for a specific treatment, and initial injection with <span class="elsevierStyleItalic">pro re nata</span> posterior administration appears to be adequate according to some authors.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">3</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall">Clinically, funduscopic examination reveals loss of retinal transparency in the juxtafoveal temporal area. In advanced stages, a visible dilatation of juxtafoveal retinal capillaries occurs,<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">7</span></a> with the additional possibility of hyper-refringent crystalline deposits located at the level of the vitreoretinal interface,<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">6</span></a> as well as right-angle dilated venules<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">9</span></a> and pigment changes such as hyperplasia plates in the RPE and/or intraretinal pigment migration.<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">7</span></a> These typical lesions were observed in the patients of the present study.</p><p id="par0130" class="elsevierStylePara elsevierViewall">Technological developments have improved knowledge of retinal diseases.<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">4,10,11</span></a> Spectral domain OCT enables diagnosing MacTel 2 cases at earlier and asymptomatic stages.<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">4</span></a> Tomographic images show diminished thickness, temporal foveal and perifoveal thinning with loss of neurosensory retina tissue, giving rise to pseudocysts in the internal as well as external layers of the retina. These findings were previously described in numerous studies.<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9,12–14</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">It would be important to emphasize the necessity of following up these patients due to the possibility of developing neovascularization and subsequent vision impairment requiring treatment. The authors recommend utilizing OCT for follow-up.</p><p id="par0140" class="elsevierStylePara elsevierViewall">As can be observed in the autofluorescence images, initially it can increase (hyperautofluorescence) due to the loss of luteal pigment<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">15</span></a> prior to clinic and angiographic changes.<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">16</span></a></p><p id="par0145" class="elsevierStylePara elsevierViewall">It has been suggested that macular pigment plays an important role in the physiopathology of several diseases, and diminished amounts of lutein and zeaxanthin have been described in the foveal area of patients with MacTel 2.<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">17</span></a> Macular pigment density distribution has been calculated with autofluorescence, identifying abnormal paracentral distribution.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">18</span></a> The central area exhibits reductions whereas the peripheral area is preserved, which could indicate a local defect in the ability to accumulate pigment.<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">18</span></a> It has not been demonstrated that supplements with lutein and zeaxanthin are able to modify the course of the disease.</p><p id="par0150" class="elsevierStylePara elsevierViewall">Infrared reflectance produces a characteristic finding of a hyporeflective perimacular ring and hyper-hyporeflectance areas matching pseudocysts and RPE hyperplasia, in addition to small refractile crystals.<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">19</span></a></p><p id="par0155" class="elsevierStylePara elsevierViewall">In fluorescein angiography, perifoveal ectasic dilated capillaries can produce exudation. In early stages, little or no evidence of capillary dilatation can be seen. In later stages, perifoveal temporal retina staining is observed, which usually respects the central foveal area. As the disease progresses, alterations completely encircle the fovea.<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9,20</span></a> In the patients of this study, leak areas were observed mainly in the temporal parafoveal area, a characteristic previously described by Thorell et al.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">9</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">OCT-A provides detailed images of retinal microcirculation. In the case of this pathology, vessel patency alterations are observed such as ectasic vessels with diffused or sectorial shape, distorted or even with neovascularization. The most frequent finding is vascular dilatation visible in the deep capillary plexus, which can progress and compromise the superficial capillary plexus.<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">9,21</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">Limitations of the present study include its retrospective nature and the small number of cases. It would be convenient to study the evolution of this disease in longer terms.</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Conflict of interests</span><p id="par0170" class="elsevierStylePara elsevierViewall">No conflict of interests was declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres993654" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Objective" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec957092" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres993655" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec957091" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Material and methods" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Results" "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Epidemiological characteristics" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Functional characteristics" ] 2 => array:2 [ "identificador" => "sec0030" "titulo" => "Phenotype characteristics in color retinography" ] 3 => array:2 [ "identificador" => "sec0035" "titulo" => "Morphometric tomographic characteristics" ] 4 => array:2 [ "identificador" => "sec0040" "titulo" => "Phenotype characteristics in ocular fundus autofluorescence" ] 5 => array:2 [ "identificador" => "sec0045" "titulo" => "Phenotype characteristics in infrared reflectance" ] 6 => array:2 [ "identificador" => "sec0050" "titulo" => "Phenotypic characteristics in fluorescein angiography" ] 7 => array:2 [ "identificador" => "sec0055" "titulo" => "Phenotypic characteristics in optic coherence tomography angiography" ] ] ] 7 => array:2 [ "identificador" => "sec0060" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0065" "titulo" => "Conflict of interests" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-07-30" "fechaAceptado" => "2017-08-23" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec957092" "palabras" => array:6 [ 0 => "Type 2 macular telangiectasia" 1 => "OCT" 2 => "OCT angiography" 3 => "Autofluorescence" 4 => "Angiography" 5 => "Prevalence" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec957091" "palabras" => array:6 [ 0 => "Telangiectasia macular" 1 => "OCT" 2 => "Angio-OCT" 3 => "Autofluorescencia" 4 => "Angiografía" 5 => "Prevalencia" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">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.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">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.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">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.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">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 (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>.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.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Objective" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Objetivo</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Las telangiectasias maculares idiopáticas de tipo<span class="elsevierStyleHsp" style=""></span>2 (MacTel<span class="elsevierStyleHsp" style=""></span>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.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Estudio retrospectivo en el que se analizaron 12 ojos de 6 pacientes con MacTel<span class="elsevierStyleHsp" style=""></span>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<span class="elsevierStyleHsp" style=""></span>meses.</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">La prevalencia de MacTel<span class="elsevierStyleHsp" style=""></span>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<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0,492). Tres pacientes desarrollaron neovascularización coroidea (NVC), requiriendo tratamiento intravítreo con agentes antiangiogénicos.</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">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<span class="elsevierStyleHsp" style=""></span>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.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Bayón-Porras RM, Pascual-Camps I, Plaza-Laguardia C, Gallego-Pinazo R. Telangiectasias maculares idiopáticas de tipo 2: prevalencia en nuestra área y estudio morfométrico y fenotípico. Arch Soc Esp Oftalmol. 2018;93:105–112.</p>" ] ] "multimedia" => array:6 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1395 "Ancho" => 1867 "Tamanyo" => 186622 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Ocular fundus photograph showing loss of transparency in the juxtafoveal region (A), right-angle vessels (B), RPE with hyperplasia (C) and crystalline deposits (D).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1091 "Ancho" => 1867 "Tamanyo" => 297963 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Macular OCT image showing typical lesions: hypo-reflective spaces and outer layer disruption in top images (A and B). Image C shows preserved outer layers, while D shows a case with choroidal nail vascularization.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1391 "Ancho" => 1867 "Tamanyo" => 403891 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Macular autofluorescence image showing the loss of macular hypoautoflurescence (A and B) and RPE hyperplasia (C and D).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1582 "Ancho" => 1867 "Tamanyo" => 303165 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Macular infrared reflectance image showing hypo-reflective perimacular ring and hyper-reflectance areas corresponding to RPE hyperplasia area (D).</p>" ] ] 4 => array:7 [ "identificador" => "fig0025" "etiqueta" => "Fig. 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 1870 "Ancho" => 1867 "Tamanyo" => 354835 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Fluorescein angiography image in early times (A and B) and late times (C and D) in a patient without CNV (C) and another with CNV (D).</p>" ] ] 5 => array:7 [ "identificador" => "fig0030" "etiqueta" => "Fig. 6" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr6.jpeg" "Alto" => 650 "Ancho" => 1000 "Tamanyo" => 183140 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">OCT-A image showing vascular dilatation with anomalous vascular anastomosis and increased intravascular spaces in the parafoveal temporal area.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:21 [ 0 => array:3 [ "identificador" => "bib0110" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Idiopathic macular telangiectasia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "L.A. 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| Year/Month | Html | Total | |
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| 2024 October | 15 | 0 | 15 |
| 2024 September | 29 | 0 | 29 |
| 2024 August | 32 | 1 | 33 |
| 2024 July | 13 | 0 | 13 |
| 2024 June | 8 | 0 | 8 |
| 2024 May | 6 | 0 | 6 |
| 2024 April | 11 | 1 | 12 |
| 2024 March | 17 | 0 | 17 |
| 2024 February | 27 | 2 | 29 |
| 2024 January | 25 | 0 | 25 |
| 2023 December | 28 | 0 | 28 |
| 2023 November | 43 | 1 | 44 |
| 2023 October | 88 | 2 | 90 |
| 2023 September | 39 | 1 | 40 |
| 2023 August | 39 | 0 | 39 |
| 2023 July | 53 | 0 | 53 |
| 2023 June | 26 | 0 | 26 |
| 2023 May | 46 | 0 | 46 |
| 2023 April | 32 | 2 | 34 |
| 2023 March | 34 | 0 | 34 |
| 2023 February | 38 | 0 | 38 |
| 2023 January | 29 | 1 | 30 |
| 2022 December | 32 | 0 | 32 |
| 2022 November | 45 | 2 | 47 |
| 2022 October | 34 | 0 | 34 |
| 2022 September | 37 | 0 | 37 |
| 2022 August | 36 | 0 | 36 |
| 2022 July | 18 | 0 | 18 |
| 2022 June | 16 | 2 | 18 |
| 2022 May | 11 | 2 | 13 |
| 2022 April | 18 | 2 | 20 |
| 2022 March | 7 | 2 | 9 |
| 2022 February | 6 | 0 | 6 |
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| 2021 December | 1 | 0 | 1 |
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| 2020 September | 0 | 1 | 1 |
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