Cilioretinal Artery Occlusion in a Young Patient with Flammer Syndrome and Increased Retinal Venous Pressure

 

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Background

Cilioretinal arteries stem from the posterior ciliary artery system and are found in roughly 32 % of all eyes [1]. In 18.7 % of the patients, a ciliary artery contributes to macular circulation [1]. In a large series of central artery occlusions, the conservation of some degree of macular function due to the existence of a cilioretinal artery could be observed in 26 % of cases [2]. The opposite phenomenon, an occlusion of a cilioretinal artery (CLRAO), represents only 5 % of all retinal arterial occlusions [3], [4] but may be associated with a significant loss of central vision [3]. The median age of 49 years [3] is lower than that of other groups of retinal vascular occlusion. CLRAOs in very young patients under the age of 25 years are rare and have been described in conjunction with a wide array of general conditions, including cocaine use [5], increased intracranial pressure [6], Sturge-Weber-associated glaucoma [7], hypercoagulable states, and cardiac valvular diseases [4]. The concurrence of a CLRAO with a central retinal vein occlusion (CRVO) was first described in 1968 [8]. CLRAOs were assumed to be secondary to CRVO [9], [10]. The proposed mechanism is a resulting reduction of the pressure gradient in the cilioretinal artery [8], [9]. We report a CLRAO in a young man with very high retinal venous pressure (RVP). Whereas this patient had neither classical vascular risk factors nor identifiable sources of emboli, he had signs of Flammer Syndrome [11], [12], [13]. The vasospastic syndrome (later called primary vascular dysregulation syndrome and today Flammer syndrome) has already been reported to predispose to both CRVO and CLRAO [14]. The increase of RVP, which in extreme cases leads to the clinical picture of a CRVO, has been described as a consequence of a local dysregulation of the central retinal vein [15]. Here we describe a case with CLRAO in the presence of very high RVP and we hypothesize that Flammer syndrome caused both a local constriction of the cilioretinal artery and of the central retinal vein. The latter increased RVP and thereby contributed to the pathogenesis of CLRAO.

• References

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