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provided by RERO DOC Digital Library Graefe’s Arch Clin Exp Ophthalmol (2005) 243: 834–836 CASE REPORT

DOI 10.1007/s00417-005-1123-z

Marc Zaninetti Cavernous elicited Alexandros N. Stangos German Abdo by a central retinal venous stasis Constantin J. Pournaras retinopathy

Abstract Background: Central reti- an atypical cavernous sinus thrombo- Received: 30 September 2004 Revised: 19 December 2004 nal vein occlusion is a relatively sis of undetermined origin. The pa- Accepted: 20 December 2004 common retinal disorder in the el- tient had experienced in the past 6 Published online: 9 March 2005 derly, and those with cardiovascular months intermittent diplopia and an # Springer-Verlag 2005 or thrombophilic risk factors are at irreducible conjunctival hyperemia. increased risk. Although still unsatis- Hemodilution was dismissed. Soon fying, some treatments for the acute after initiation of anticoagulation ’ No financial interest. No grant. and chronic phases have been estab- therapy, the patient s clinical signs lished based on randomized studies. and symptoms improved. Final visual M. Zaninetti . A. N. Stangos However, for rare conditions mim- acuity was 0.8. Conclusion: Venous Hôpital Ophtalmique Jules-Gonin, icking occlusion, stasis retinopathy secondary to cav- 1004 Lausanne, Switzerland treatment of the acute phase should be ernous sinus thrombosis is rare. G. Abdo targeted at etiology. A rare condition However, careful clinical examination Département de radiodiagnostic, mimicking central retinal vein occlu- and extensive laboratory work-up is Hôpitaux Universitaires Genevois, sion in a 70-year-old man is presented needed to exclude central retinal vein Geneva, Switzerland and discussed. Methods: A 70-year- occlusion not associated with com- C. J. Pournaras (*) old man was admitted to the hospital mon vascular pathologies of the Clinique d’ophtalmologie, for isovolemic hemodilution related to elderly. Hôpitaux Universitaires Genevois, a central retinal vein occlusion of the 22, rue Alcide-Jentzer, elderly, after a sudden decrease in Keywords Central retinal vein 1211 Geneva 14, Switzerland . . e-mail: [email protected] visual acuity to 0.1. Results: Clinical occlusion Carotid-cavernous fistula Tel.: +41-22-3828394 and laboratory work-up demonstrated Hemodilution . Arteriography . Fax: +41-22-3828421 a venous stasis retinopathy, related to Anticoagulation . Proptosis

Introduction for acute stages of CRVO [4, 5], it should not be carried out until other causes of central retinal vein venous stasis ret- In the elderly, the principal risk factors for central retinal inopathy can be reasonably discounted, because of the vein occlusion (CRVO) are arterial hypertension, diabetes possible risk of aggravating the condition, especially in the mellitus and intraocular hypertonia [9]. However, central case of a carotid-cavernous fistula. retinal vein venous stasis retinopathy mimicking CRVO occasionally results from disease affecting the venous drainage such as retinal arteriovenous communications, Case report carotid-cavernous fistula, cavernous sinus thrombosis or intraorbital processes. All the above leads to an increase of We report the case of a 70-year-old man with neither intraluminal pressure within the central retinal vein and cardiovascular nor thrombophilic risk factors who was re- consecutive venous stasis at the peripheral retinal venous ferred to the clinic by his ophthalmologist for isovolemic circulation. Although lowering blood viscosity and admin- hemodilution for CRVO. He mainly complained of a re- istration of antiaggregating agents is the standard treatment cently sudden loss of visual acuity of the right eye (OD); 835

14 mmHg OS). The OD fundus presented with tortuous and dilated vessels, intraretinal hemorrhages (Fig. 1a) and a macular edema. The arteriovenous time was severely de- layed at fluorescein angiography. The was of normal caliber on ultrasound. Orthoptic tests indicated a mechanical limitation of extraocular muscles, but without any nerve palsies. Laboratory testing was nega- tive for any thrombophilic disorders. At this time, it was decided to dismiss the isovolemic hemodilution because the clinical appearance strongly suggested a carotid-cavernous fistula or an intraorbital process, and orbital and cerebral Fig. 1 a Fluorescein angiography of the right eye showing pre- imaging was ordered. CT imaging showed a right orbital dominating tortuous and dilated retinal in the acute phase. blood stasis without mass effect, whereas angio-MRI showed b Fluorescein angiography of the right eye showing regression of tortuous and dilated retinal veins and hemorrhages in the remitting a delayed right cavernous sinus perfusion. Moreover, ce- phase rebral arteriography showed lack of opacification of the right sinus cavernosus, yielding a right cavernous sinus thrombosis as the probable diagnosis (Fig. 2a1–2, b1–2). he had, however, experienced in the past 6 months two Since no imaging techniques could elicit a carotid-cavern- episodes of painless diplopia, each one lasting less than 2 ous fistula, we considered it as excluded. A low-molecular- weeks. These episodes were attributed to sinusitis by a weight heparin anticoagulation promptly followed by a general ophthalmologist. The patient was also concerned coumarin anticoagulation with INR between 2.5 and 3.5 by a recurrent conjunctival hyperemia resistant to anti-in- was immediately started by the neurologist. In a few weeks, flammatory drops. No orbital murmur could be retrieved the conjunctival/episcleral vessel dilation regressed and the from anamnestic data and clinical examination. The exam- fundus clinical picture returned to almost normal (Fig. 1b), ination revealed visual acuity (VA) OD of 0.1, a striking except for the persistence of a moderate macular edema, dilation of the conjunctival/episcleral vessels, proptosis which vanished after a single intravitreal administration of (Hertel: 13-109-9) and a slowly reactive pupil. The intra- triamcinolone. VA OD, however, remained low because ocular pressure was slightly asymmetrical (17 mmHg OD; of an advancing cataract, but increased to 0.8 after cataract

Fig. 2 a Arteriography showing injection of contrast medium into the left common carotid (1 lateral, 2 anteroposte- rior). Note the opacification of the left cavernous sinus (ar- rows). b Arteriography showing injection of contrast medium into the right common carotid artery (1 lateral, 2 anteroposte- rior). Note the lack of opacifi- cation of the right cavernous sinus (arrows) 836 extraction 2 months later. At the same time, angio-MRI nous hypertension, generating signs and symptoms related showed no delay anymore of the right sinus cavernous to ischemia or tissue engorgement. Finally, after a time lapse perfusion. After a 4-month course, the anticoagulation was of orbital venous hypertension, the blood–retina barrier stopped and replaced by low-dose aspirin. broke down, causing a central retinal vein venous stasis retinopathy. A number of cases of central retinal vein ve- nous stasis retinopathy have been described after carotid- Discussion cavernous fistula (e.g., [3, 7]), far more often than after cavernous sinus thrombosis. However, venous sinus hy- Several reports discuss the occurrence of cavernous sinus pertension and venous sinus thrombosis may lead to indi- thrombosis associated with CRVO [1, 2, 8]. However, the rect [6]. One can thus imagine that case presented here differs in several aspects from the pre- cavernous sinus thrombosis of the type presented here vious reports and from classical cavernous sinus throm- could have occurred more often, but would have evolved bosis. A classical cavernous sinus thrombosis has an acute before diagnosis into an indirect carotid-cavernous fistula, onset which may be accompanied by an alteration of con- or alternatively the thrombosis would have spontaneously sciousness. Even if the symptoms are milder, a precipitating resolved. event can generally be found, either a contiguous infection Isovolemic hemodilution is the proposed treatment for or a head trauma or head surgery within a few days to weeks the acute phase of CRVO. However, in conditions mim- before. Our patient, however, developed varying symptoms icking CRVO but not being classical CRVO, this treatment at varying intervals, no one symptom being at first sug- has not been studied. Even if it could be relatively safe in gestive of a cavernous sinus thrombosis, and denied any central retinal vein venous stasis retinopathy caused by a previous head trauma. Despite this very atypical history, the cavernous sinus thrombosis, it would, however, possibly diagnosis of cavernous sinus thrombosis remains likely for be thought unsafe in the case of a carotid-cavernous fis- the following reasons: CTand MRI showed an orbital blood tula, since the hemodynamic state would allow increasing engorgement without any other tissue abnormalities, thus flow into the fistula. In the case presented here, postponing the process was purely intravascular. Extensive serological hemodilution was of prime importance since the suspicion and blood testing did not yield results positive for sar- of carotid-cavernous fistula was primarily high. coidosis and other orbital or cavernous sinus diseases. Ce- Although rare in the elderly, central retinal vein venous rebral arteriography, corroborating MRI angiographic stasis retinopathy of this type should not be confused with sequences, showed a lack of perfusion of the right cavern- a classical CRVO, since the treatment of the acute phase ous sinus. Lastly, the patient began to recover quickly after might be targeted according to the etiology. It is thus of the introduction of an anticoagulation therapy. importance to carry out a careful clinical examination and, We thus conjecture that a previous sinus infection caused if needed, extensive laboratory work-up for untypical his- some months earlier a low-grade septic cavernous sinus tory of CRVO. thrombosis, which evolved into a predominant orbital ve-

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