{
    "abstract": "To explore the clinical and multimodal imaging characteristics of Intraretinal Fibrosis (IRFib) in Macular Telangiectasia Type-2 (MacTel).\nMacTel eyes with IRFib that had multimodal imaging with color fundus photography, multicolor (MC), blue reflectance, spectral-domain optical coherence tomography (OCT), OCT-angiography (OCTA) and fluorescein angiography.\nSeven eyes of 6 patients were included. Clinically, all cases had grayish-white fibrosis at macula, with retinal vessel tortuosity in two cases best characterized on MC. Two eyes each belonged to Gass and Blodi stages 3 and 4, three eyes were stage 5 MacTel. The mean lesion area at presentation was 1.36 \u00b1 0.81 mm2 (range 0.56-2.99 mm2). The defining OCT finding for IRFib was hyper-reflective distortion intraretinally (involving different layers). Other accompanying findings in decreasing order were collapse sign (7 eyes); epiretinal membrane (6 eyes); pigment clumping and migration (5 eyes); serrated internal limiting membrane and outer-retinal hyper-reflectivity (4 eyes); and lamellar macular hole (1 eye). On OCTA, IRFib was associated with right-angled vessels, vascular invasion and distortion of foveal avascular zone (FAZ), and vessel dilatation and tortuosity in superficial vascular complex; telangiectasia, FAZ distortion and invasion, vessel drag, capillary looping, and abnormal vascular structures in deep capillary plexus; and nodular tuft or twig-like hyper-reflective vascular lesions in outer-retina choriocapillaris slab.\nThe incidence of IRFib on MC in our MacTel cohort was 1.75% with characteristic findings on OCT and OCTA. They are associated with advanced MacTel and probably implicate M\u00fcller cell dysfunction, activated glial cells, and vascular changes during the disease process.",
    "authors": [
        {
            "affiliation": "Department of Vitreoretinal Services, Giridhar Eye Institute, Cochin, Kerala, India.\nSSM Eye Research Foundation, Giridhar Eye Institute, Cochin, Kerala, India.",
            "firstname": "Kiran",
            "initials": "K",
            "lastname": "Chandran"
        },
        {
            "affiliation": "Department of Vitreoretinal Services, Giridhar Eye Institute, Cochin, Kerala, India.\nSSM Eye Research Foundation, Giridhar Eye Institute, Cochin, Kerala, India.",
            "firstname": "Anantharaman",
            "initials": "A",
            "lastname": "Giridhar"
        },
        {
            "affiliation": "Department of Vitreoretinal Services, Giridhar Eye Institute, Cochin, Kerala, India.",
            "firstname": "Mahesh",
            "initials": "M",
            "lastname": "Gopalakrishnan"
        },
        {
            "affiliation": "NIHR Biomedical Research Centre, Moorfields Eye Hospital, NHS Foundation Trust, London, United Kingdom.",
            "firstname": "Sobha",
            "initials": "S",
            "lastname": "Sivaprasad"
        }
    ],
    "conclusions": "The incidence of IRFib on MC in our MacTel cohort was 1.75% with characteristic findings on OCT and OCTA. They are associated with advanced MacTel and probably implicate M\u00fcller cell dysfunction, activated glial cells, and vascular changes during the disease process.",
    "copyrights": null,
    "doi": "10.1097/ICB.0000000000001768",
    "journal": "Retinal cases & brief reports",
    "keywords": [
        "Contraction",
        "Intraretinal Fibrosis (IRFib)",
        "Macular Telangiectasia Type-2 (MacTel)",
        "Multicolor (MC)",
        "Optical coherence tomography (OCT)",
        "Optical coherence tomography angiography (OCTA)"
    ],
    "methods": null,
    "publication_date": "2025-04-25",
    "pubmed_id": "40279574",
    "results": "Seven eyes of 6 patients were included. Clinically, all cases had grayish-white fibrosis at macula, with retinal vessel tortuosity in two cases best characterized on MC. Two eyes each belonged to Gass and Blodi stages 3 and 4, three eyes were stage 5 MacTel. The mean lesion area at presentation was 1.36 \u00b1 0.81 mm2 (range 0.56-2.99 mm2). The defining OCT finding for IRFib was hyper-reflective distortion intraretinally (involving different layers). Other accompanying findings in decreasing order were collapse sign (7 eyes); epiretinal membrane (6 eyes); pigment clumping and migration (5 eyes); serrated internal limiting membrane and outer-retinal hyper-reflectivity (4 eyes); and lamellar macular hole (1 eye). On OCTA, IRFib was associated with right-angled vessels, vascular invasion and distortion of foveal avascular zone (FAZ), and vessel dilatation and tortuosity in superficial vascular complex; telangiectasia, FAZ distortion and invasion, vessel drag, capillary looping, and abnormal vascular structures in deep capillary plexus; and nodular tuft or twig-like hyper-reflective vascular lesions in outer-retina choriocapillaris slab.",
    "title": "Intraretinal Fibrosis in Macular Telangiectasia Type-2 (MacTel): Clinical and Multimodal Imaging Features.",
    "xml": "<Element 'PubmedArticle' at 0x788506ed0fe0>"
}