Isolated patchy perivenular whitening: When white edema of the posterior pole may be vascular in origin but not a retinal artery occlusion

To identify cases of non-ischemic retinal vein occlusion (RVO) presenting as isolated patchy perivenular retinal whitening and to describe diagnostic considerations and short-term natural history. Retrospective observational monocentric case series. Patients monitored in one center between January 2013 and January 2015. Among the 151 patients monitored in our center for retinal vein occlusion, we identified patients presenting with isolated patchy perivenular whitening revealing retinal venous occlusion. Eight patients presenting with isolated perivenular whitening revealing RVO were identified (3.9 %). There were five central retinal vein occlusions, three hemi-retinal vein occlusions and no branch retinal vein occlusions. Initial visual acuity was preserved in five out of eight cases. In all cases, patchy perivenular whitening was isolated, without retinal hemorrhage. Blue monochromatic photographs allowed visualization of patchy perivenular whitening in all cases. Optical coherence tomography (OCT) showed a focal and segmental hyper-reflective area in the inner retinal layers, especially in the inner nuclear layer. One month after diagnosis, atrophy was noted in the retinal layers showing initial hyper-reflectivity. Three cases of our series of eight were initially mistakenly diagnosed as central retinal arterial occlusion. The main complication of perivenular whitening is the atrophy of inner retinal layers responsible for paracentral scotomas. The differential diagnosis with retinal arterial occlusion, which may be difficult, is based on the angiographic and OCT features, and their progression.