Abstract：Objective: To explore the imaging characteristics of a multi-mode imaging techniques at different stages of Stargardt's disease. Methods: This was a retrospective analysis of 28 cases (56 eyes) of patients with Stargardt's disease diagnosed in Affiliated Hospital of Yunnan University from May 2016 to August 2019. Fundus color photography, autofluorescence, fundus fluorescein angiography, optical coherence tomography (OCT), multicolor fundus imaging, OCT angiography (OCTA), and characteristics of various imaging examinations at different stages of the disease were summarized. Results: Color photography of the fundus showed that the atrophic lesions in the macular area gradually expand with the progression of the lesion, with color change at the posterior pole. Multicolor imaging combines multiple wavelengths to show the atrophy of cells and range with better contrast. The fundus autofluorescence showed the damage in the retinal pigment epithelium (RPE), and can clearly and visually show the size of the atrophy lesion in the macular area. Fundus fluorescein angiography can directly reflect the degree of damage in the retinal pigment epithelial layer and choroidal blood vessels in Stargardt's disease. With the development of Stargardt's disease, the atrophy of the RPE layer in the macular area was enlarged, and the retina and choroidal capillaries progressively atrophied. OCT provided image information of the fundus in different layers. As the lesion progressed, the peripheral nerve epithelial layer became thinner, the photoreceptors and RPE shrank and disappeared to varying degrees, and the choroidal capillary shrank from the depth and range of the layer. In the OCTA, we observed that as the lesion progressed, the vascular density at each level decreased. When the deep blood vessels of the retina were scanned, the choroidal blood vessels below were observed, and as the atrophy increased, the range of the exposed choroidal vessels increased.
Tsang SH, Sharma T. Stargardt Disease. Adv Exp Med Biol,2018, 1085: 139-151 DOI: 10.1007/978-3-319-95046-4_27.
[2]
Hussain RM, Ciulla TA, Berrocal AM, et al. Stargardt macular dystrophy and evolving therapies. Expert Opin Biol Ther, 2018,18(10): 1049-1059. DOI: 10.1080/14712598.2018.1513486.
[3]
Gomes NL, Greenstein VC, Carlson JN, et al. A comparison of fundus autofluorescence and retinal structure in patients with Stargardt disease. Invest Ophthalmol Vis Sci, 2009, 50(8): 3953-3959. DOI: 10.1167/iovs.08-2657.
[4]
Lambertus S, van Huet RA, Bax NM, et al. Early-onset stargardt disease: phenotypic and genotypic characteristics. Ophthalmology, 2015, 122(2): 335-344. DOI: 10.1016/j.ophtha.2014.08.032.
[5]
Han Z, Conley SM, Naash MI. Gene therapy for Stargardt disease associated with ABCA4 gene. Adv Exp Med Biol, 2014,801: 719-724. DOI: 10.1007/978-1-4614-3209-8_90.
Fujinami K, Zernant J, Chana RK, et al. Clinical and molecular characteristics of childhood-onset Stargardt disease.Ophthalmology, 2015, 122(2): 326-334. DOI: 10.1016/j.ophtha.2014.08.012.
[8]
Bird AC, Phillips RL, Hageman GS. Geographic atrophy: a histopathological assessment. JAMA Ophthalmol, 2014, 132(3):338-345. DOI: 10.1001/jamaophthalmol.2013.5799.
[9]
Pang CE, Freund KB. Ghost maculopathy: an artifact on near-infrared reflectance and multicolor imaging masquerading as chorioretinal pathology. Am J Ophthalmol, 2014, 158(1): 171-178.e2. DOI: 10.1016/j.ajo.2014.03.003.
[10]
Smith RT, Gomes NL, Barile G, et al. Lipofuscin and autofluorescence metrics in progressive STGD. Invest Ophthalmol Vis Sci, 2009, 50(8): 3907-3914. DOI: 10.1167/iovs.08-2448.
[11]
Freund KB, Mrejen S, Jung J, et al. Increased fundus autofluorescence related to outer retinal disruption. JAMA Ophthalmol, 2013, 131(12): 1645-1649. DOI: 10.1001/jamaophthalmol.2013.5030.
[12]
Kurz-Levin MM, Halfyard AS, Bunce C, et al. Clinical variations in assessment of bull's-eye maculopathy. Arch Ophthalmol, 2002, 120(5): 567-575. DOI: 10.1001/archopht.120.5.567.
[13]
Greenberg JP, Sherman J, Zweifel SA, et al. Spectral-domain optical coherence tomography staging and autofluorescence imaging in achromatopsia. JAMA Ophthalmol, 2014, 132(4):437-445. DOI: 10.1001/jamaophthalmol.2013.7987.
[14]
Lee JY, Lee DH, Lee JY, et al. Correlation between subfoveal choroidal thickness and the severity or progression of nonexudative age-related macular degeneration. Invest Ophthalmol Vis Sci, 2013, 54(12): 7812-7818. DOI: 10.1167/iovs.13-12284.
[15]
Voigt M, Querques G, Atmani K, et al. Analysis of retinal flecks in fundus flavimaculatus using high-definition spectral-domain optical coherence tomography. Am J Ophthalmol, 2010, 150(3):330-337. DOI: 10.1016/j.ajo.2010.04.001.
[16]
Battaglia Parodi M, Cicinelli MV, Rabiolo A, et al.Vascular abnormalities in patients with Stargardt disease assessed with optical coherence tomography angiography.Br J Ophthalmol, 2017, 101(6): 780-785. DOI: 10.1136/bjophthalmol-2016-308869.
[17]
Mastropasqua R, Toto L, Borrelli E, et al. Optical coherence tomography angiography findings in Stargardt disease.PLoS One, 2017, 12(2): e0170343. DOI: 10.1371/journal.pone.0170343.
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