Subscribe to RSS
DOI: 10.1055/s-0035-1545774
Cilioretinal Artery Occlusion in a Young Patient with Flammer Syndrome and Increased Retinal Venous Pressure
Verschluss einer zilioretinalen Arterie bei einem jungen Patienten mit Flammer-Syndrom und erhöhtem retinalen VenendruckPublication History
received 00.00.00
accepted 00.00.00
Publication Date:
22 April 2015 (online)
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
- 1 Justice jr. J, Lehmann RP. Cilioretinal arteries. A study based on review of stereo fundus photographs and fluorescein angiographic findings. Arch Ophthalmol 1976; 94: 1355-1358
- 2 Brown GC, Shields JA. Cilioretinal arteries and retinal arterial occlusion. Arch Ophthalmol 1979; 97: 84-92
- 3 Stoffelns BM. [Isolated cilioretinal artery occlusion – clinical findings and outcome in 31 cases]. Klin Monatsbl Augenheilkd 2012; 229: 338-342
- 4 Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol 1995; 120: 776-783
- 5 Kannan B, Balaji V, Kummararaj S et al. Cilioretinal artery occlusion following intranasal cocaine insufflations. Indian J Ophthalmol 2011; 59: 388-389
- 6 McCannel CA. Choroidal infarction or cilioretinal artery occlusion in the setting of elevated intracranial pressure due to fulminant idiopathic intracranial hypertension?. J Neuroophthalmol 2011; 31: 194-195
- 7 Chang L, Mruthyunjaya P, Rodriguez-Rosa RE et al. Postoperative cilioretinal artery occlusion in Sturge Weber-associated glaucoma. J AAPOS 2010; 14: 358-360
- 8 Zhang L, Sun Y, Johnson MW. Combined cilioretinal artery and central vein occlusions in juvenile glaucoma. Arch Ophthalmol 2011; 129: 1231-1234
- 9 Schatz H, Fong AC, McDonald HR et al. Cilioretinal artery occlusion in young adults with central retinal vein occlusion. Ophthalmology 1991; 98: 594-601
- 10 Hayreh SS, Fraterrigo L, Jonas J. Central retinal vein occlusion associated with cilioretinal artery occlusion. Retina 2008; 28: 581-594
- 11 Konieczka K, Ritch R, Traverso CE et al. Flammer syndrome. EPMA J 2014; 5: 11
- 12 Flammer J, Konieczka K, Flammer AJ. The primary vascular dysregulation syndrome: implications for eye diseases. EPMA J 2013; 4: 14
- 13 Flammer J, Konieczka K, Bruno RM et al. The eye and the heart. Eur Heart J 2013; 34: 1270-1278
- 14 Flammer J, Pache M, Resink T. Vasospasm, its role in the pathogenesis of diseases with particular reference to the eye. Prog Retin Eye Res 2001; 20: 319-349
- 15 Fraenkl SA, Mozaffarieh M, Flammer J. Retinal vein occlusions: The potential impact of a dysregulation of the retinal veins. EPMA J 2010; 1: 253-261