A 17-year-old male patient diagnosed with Williams-Beuren syndrome presented with a gradual decline in vision in both eyes during a 3-year period. The ophthalmologic examination was notable for numerous loop-shaped tortuous vessels accompanying normal appearing retinal vessels and cystoid macular edema (CME). Mild foveal hypoplasia with a persistence of inner retinal layers was noted on spectral-domain optical coherence tomography (OCT). OCT angiography showed that the abnormal vessels were not retinal vessels but were compatible with prepapillary vascular loops. The CME persisted despite repeated intravitreal bevacizumab treatment. The resolution was observed following an intravitreal triamcinolone acetonide injection. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:514-518.].
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