Central retinal vein occlusion with cilioretinal infarction from branch flow exclusion and choroidal arterial steal

The first definitive study of retinal vein occlusion complicated by infarction within the territory of one or more cilioretinal arteries was published in 1976. Many individual cases and further case series have been reported in the interim, but the nature of the interrelationship is still under debate.

A review was undertaken of the relevant clinical and fundus fluorescein angiographic characteristics of this combined retinal vascular disorder together with the pathophysiological mechanisms currently presented in the literature to explain their association. Scientific publications up to 2008 were evaluated by one of the authors of the original report.

There are broad similarities between publications in their descriptions of the clinical features, but significant differences of detail and interpretation are also evident. Most of the mechanisms so far proposed to account for cilioretinal infarction after central or hemisphere retinal vein occlusion do not withstand critical scrutiny. Two related hypotheses are expounded that appear to satisfactorily elucidate this interrelationship -- branch flow exclusion and branch flow diversion (otherwise termed "choroidal arterial steal").

In eyes with a cilioretinal supply, the probability that cilioretinal infarction will complicate retinal vein occlusion increases with increasing severity of venous obstruction and the more distally the cilioretinal artery arises from the posterior ciliary arterial tree. A distal branch point also facilitates observation of dye front reciprocation within the artery. Indicators of the degree of venous obstruction that may be necessary to instigate cilioretinal infarction include very prolonged dye transit times in the central retinal circulation, exaggerated venous cyanosis and tortuosity, perivenous cotton-wool sentinels, and macular perivenular whitening.