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

• Published in: Archivos de la Sociedad Española de Oftalmología (English ed.) 2018-03, Vol.93 (3), p.105-112
• Main Authors: Bayón-Porras, R.M., Pascual-Camps, I., Plaza-Laguardia, C., Gallego-Pinazo, R.
• Format: Article
• Language: English
• Subjects: Aged; Angio-OCT; Angiografía; Angiography; Autofluorescence; Autofluorescencia; Diagnostic Techniques, Ophthalmological; Female; Humans; Middle Aged; OCT; OCT angiography; Phenotype; Prevalence; Prevalencia; Retinal Telangiectasis - classification; Retinal Telangiectasis - diagnostic imaging; Retinal Telangiectasis - epidemiology; Retinal Telangiectasis - genetics; Retrospective Studies; Telangiectasia macular; Type 2 macular telangiectasia
• ISSN: 2173-5794; 2173-5794
• DOI: 10.1016/j.oftale.2017.08.002

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 tipo2 (MacTel2) 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 MacTel2. 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 18meses. La prevalencia de MacTel2 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), req