Optical coherence tomography angiography analysis of fabry disease
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ORIGINAL PAPER
Optical coherence tomography angiography analysis of fabry disease Ayse Idil Cakmak . Eray Atalay . Veysel Cankurtaran . Erdog˘an Yas¸ ar . Faruk Hilmi Turgut
Received: 1 January 2020 / Accepted: 20 June 2020 Ó Springer Nature B.V. 2020
Abstract Purpose Fabry disease (FD) is characterized by a deficiency in a-galactosidase A activity that leads to the cumulative deposition of unmetabolized glycosphingolipids within organs, including the vascular endothelium and the eyes. The purpose of this study was to assess the effects of FD on the retinal microvasculature, foveal avascular zone (FAZ), macular thickness and retinal nerve fiber layer (RNFL) using optical coherence tomography angiography (OCT-A). Methods Twenty-five patients (14 female and 11 male; mean age 33.16 ± 11.44) with genetically verified FD were compared with 37 age- and sexmatched healthy controls (mean age 32.36 ± 15.54). A. I. Cakmak (&) V. Cankurtaran Tayfur Ata Sokmen Medical School, Department of Ophthalmology, Hatay Mustafa Kemal University, 31040 Antakya, Hatay, Turkey e-mail: [email protected] E. Atalay Department of Ophthalmology, Eskis¸ ehir Osmangazi University Medical School, Eskis¸ ehir, Turkey E. Yas¸ ar Department of Ophthalmology, Aksaray University Medical School, Aksaray, Turkey
The vessel density (VD) values of the superficial and deep capillary plexuses (SCP and DCP), the area of the FAZ, the density of radial peripapillary capillaries (RPC), the macular thickness and the retinal nerve fiber layer thickness were measured by OCT-A examination. Results The patients showed significantly lower VD values than controls in the foveal regions of both SCP and the DCP (21.15 ± 5.56 vs. 23.79 ± 4.64 (p = 0.048), 37.92 ± 6.78 vs. 41.11 ± 5.59 (p = 0.048), respectively). The FAZ was significantly larger in the FD group than in the control group (0.3 ± 0.1 vs. 0.24 ± 0.08 (p = 0.011)). No significant difference was identified in measurements of RPC density, peripapillary RNFL thickness or macular thickness between the two groups (p [ 0.05 for all). Conclusion Decreased VD and an enlarged foveal avascular area suggest possible changes in the retinal microvasculature of patients with FD. OCT-A can serve as a useful, noninvasive, quantitative tool for diagnosing FD and monitoring its progression. Keywords Fabry disease Optical coherence tomography angiography Retinal vessel density Radial peripapillary capillaries Peripapillary retinal nerve fiber layer
F. H. Turgut Tayfur Ata Sokmen Medical School, Department of Internal Medicine, Nephrology Clinics, Hatay Mustafa Kemal University, Antakya, Hatay, Turkey
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Int Ophthalmol
Introduction Fabry disease (FD) is an X-linked inherited disorder characterized by a deficiency in a-galactosidase A activity that leads to the cumulative deposition of unmetabolized glycosphingolipids within the lysosomes of various tissues [1–4]. This situation is believed to trigger inflammation in multiple organs, including the eyes [5, 6]. As th
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