PMC5671698_01
Right fundus photograph of a 12-year-old patient with a congenital retinal macrovessel (arrow) extending towards the fovea in a superotemporal course. The foveal reflex appears disturbed by the macrovessel.
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fig1
Examination of the right fundus revealed a healthy optic disc, with an aberrant retinal macrovessel (Figure 1) arising from the inferotemporal major vascular arcade and extending towards the fovea.
Swept-source OCT angiography (a, b, c, and d) with corresponding segmentation B scan (e, f, g, and h) and en face OCT imaging (i, j, k, and l) of the right eye of a 12-year-old patient with congenital retinal macrovessel (a, e, and i). At the level of the superficial capillary plexus the principal stem of the macrovessel and two main bifurcations are noted (+). Microvascular interconnections obliterate the foveal avascular zone (a). Corresponding SS-en face OCT does not indicate any leakage from the macrovessels (b, f, and j). At the level of the deep capillary plexus there are multiple branches emanating from the principal stem and the bifurcations directed at the level of the outer plexiform layer. The latter extend and bifurcate further horizontally prior to fusing with the capillaries of the deep vascular plexus. The remaining foveal avascular zone is also depicted (arrow), considerably reduced in extent. En face OCT also clearly demonstrates the aberrant vasculature and no evidence of leakage at this level. Scans at the level of outer retina (c, g, and k) and choriocapillaris (d, h, and l) demonstrate projection artefacts of major vessels without evidence of coexisting pathology.
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fig2
Topcon) (Figure 2) demonstrated the aberrant vessel encroaching into the foveal avascular zone (FAZ) but not passing through the center of the fovea. The anatomy of the neurosensory retina appeared preserved, despite the elevation of the foveal depression.
Swept-source OCT at the level of the right fovea (a) and inferior to the fovea (b) in a 12-year-old patient with congenital retinal macrovessels. The stem of the vessel is depicted at the level of the inner nuclear and plexiform layers (arrow) and its branches at the level of the outer nuclear layer (+). The foveal anatomy is largely preserved, though the foveal depression is reduced. There is no evidence of disturbance of the RPE-choriocapillaris complex also confirmed by OCT-A in the previous image. Corresponding swept-source OCT scans at the level of the left fovea (c) and inferior to the fovea (d) are included for comparison.
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fig3
There was no evidence of disturbance in the vitreoretinal interface and no disturbance of the RPE-choriocapillaris complex (Figure 3).