Abstract
PurposeTo evaluate macular vascular abnormalities in patients with macular dystrophies (MD) and retinitis pigmentosa (RP) through application of optical coherence tomography angiography (OCT-A).MethodsIn this retrospective study, patients with MD and RP were examined by OCT-A and compared to healthy individuals. OCT-A images were analyzed regarding the diameter and surface area of the foveal avascular zone (FAZ) as well as flow (FL) in different retinal layers (superficial vascular complex (SVC), intermediate capillary complex (ICP), deep capillary complex (DCP), choriocapillaris (CC), and choroid (CD)).ResultsTwenty-one patients with MD, 21 patients with RP without macular edema (RPnE), 8 patients with RP with edema (RPwE), and 41 healthy individuals were enrolled. The group of MD and RPnE patients showed none or only minor changes in FAZ. In RPwE patients, the FAZ was significantly smaller in vertical and horizontal measurements and surface area in SVC, whereas it was markedly enlarged in ICP. FL was significantly reduced compared to healthy individuals by an average of 13.2% in CD, 14.2% in CC, and 8.4% in DCP in all patient groups. In ICP, the reduction was 9.2% for RPnE and 12.7% for RPwE patients. The SVC showed reduced FL in the MD (8.1%) and RPnE (10.3%) group.ConclusionsOCT-A is a valuable tool to examine retinal vascular abnormalities in patients with MD and RP. OCT-A revealed a reduced flow in various retinal layers in MD, RPnE, and RPwE. Alterations of the FAZ were less distinct in these groups which add to the variation reported previously.
Highlights
Inherited retinal dystrophies (IRD) are characterized by loss of visual function due to progressive degeneration of the photoreceptor/retinal pigment epithelium complex
This study addresses the most common groups of IRD, retinitis pigmentosa (RP) [6], and macular dystrophies (MD) [7]
In the intermediate capillary plexus (ICP), the RP patients showed a significant reduction for the RPnE (9.2%) and Narrowing of larger retinal vessels has been described as a characteristic sign of RP on ophthalmoscopy [6]
Summary
Inherited retinal dystrophies (IRD) are characterized by loss of visual function due to progressive degeneration of the photoreceptor/retinal pigment epithelium complex. Graefe's Archive for Clinical and Experimental Ophthalmology group of diseases with limited therapeutic options until recently The latter is currently changing due to the development of gene therapeutic and medical treatment options [3, 4]. MD represents a very heterogeneous group of disorders with less associated genes but a higher variability of clinical presentation. Photophobia, color vision deficits, reading problems, and visual acuity loss develop early and precede visual field defects [7]. Both RP and MD may develop in association with syndromic disorders. We present alterations of the OCT-A in patients with RP or MD in comparison to a group of normal controls
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