Central retinal vein occlusion: a case report and review of the literature
© licensee BioMed Central Ltd. 2009
Received: 14 April 2009
Accepted: 16 May 2009
Published: 3 June 2009
Central retinal vein occlusion is one of the major causes of severe vision impairment and blindness in adults.
We present a case of unilateral ischemic central retinal vein occlusion in a 54-year-old woman with history of uncontrolled hypertension and open angle glaucoma. Laboratory tests including complete hypercoagulability and thrombotic workup were completed.
Our case illustrates an interesting presentation of unilateral ischemic central retinal vein occlusion, where hypertension and glaucoma were thought to be the main risk factors. Close follow up, tight blood pressure and glaucoma control are crucial to prevent similar scenario in the fellow eye.
Central retinal vein occlusion (CRVO) is one of the major causes of severe vision impairment and blindness .
Thrombosis of the central retinal vein results in venous stasis, leading to disc swelling, diffuse nerve fiber layer and pre-retinal hemorrhage, and cotton wool spots that create a dramatic appearance, often called "the blood and thunder" fundus.
The prevalence of central retinal vein occlusion (CRVO) is 0.1 to 0.7 percent in population-based studies . A population-based study found a cumulative incidence of 0.5 percent over 15 years, with a prevalence of 1.3 percent for people aged 65 years and older .
Occlusion or thrombosis of the central retinal vein is associated with chronic glaucoma, atherosclerotic risk factors (age, diabetes, and hypertension), hyperviscosity, coagulopathy, and migraine -. The cause of retinal vein occlusion is often unknown.
While vision loss may be severe, the onset is typically subacute, in contrast to the sudden visual loss typical of central retinal artery occlusion. When venous stasis is severe, infarction may occur due to slowed retinal blood flow on the arterial side. In this setting, a relative afferent papillary defect is often present.
We present an interesting case of unilateral ischemic central retinal vein occlusion. We will discuss the risk factors, the complications, and the management guidelines in CRVO.
A 54-year-old African American female with history of uncontrolled hypertension and glaucoma presented to the eye clinic with sudden vision loss in the right eye upon awakening. She has been taking Clonidine 10 mg oral twice daily and Latanoprost 1 drop in each eye twice daily.
Vital signs upon presentation are summarized in Table 1
Ophthalmologic exam showed best corrected visual acuity of hand motion in the right eye and 20/70 in the left eye. Pupil exam showed sluggish right pupil with relative afferent papillary defect, and a reactive pupil on the left.
Intraocular pressures were 19 mmHg in the right, and 11 mmHg in the left.
Slit lamp exam showed normal anterior segments with open angles bilaterally.
Laboratory tests including hypercoagulability workup are summarized in Table 2
White blood cell count
33 normal (Reference normal < 40)
Serum protein electrophoresis
Further hypercoagulability workup is summarized in Table 3
Functional protein S assay
Functional protein C assay
Functional antithrombin III assay
Antiphospholipid antibody titer
262 (reference range = 151-264)
11.5 (0-10) High
0.9 mg per deciliter
Factor V Leiden PCR assay
Patient was prescribed aspirin 81 mg daily. Amlodipine 10 mg, and Hydrochlorothiazide 25 mg were added to achieve better blood pressure control. Timolol and Brimonide eye drops were added to achieve better control of the intraocular pressure.
Patient was advised to follow closely during the next six months, including gonioscopy and undilated examination of the iris to check for neovascularization of the iris/disc.
CRVO has two types:
â€¢Â Nonischemic (70%): which is characterized by vision that is better than 20/200, 16% progress to nonperfused; 50% resolve completely without treatment; defined as <10 disk diameter (DD) of capillary nonperfusion.
â€¢Â Ischemic (30%): which is defined as more than 10 DD of nonperfusion; patients are usually older and have worse vision; 60% develop iris NV; up to 33% develop neovascular glaucoma; 10% are combined with branch retinal arterial occlusion (usually cilioretinal artery due to low perfusion pressure of choroidal system) .
Central retinal vein occlusion is a disease of the old population (age >50 years old). Major risk factors are hypertension, diabetes, and atherosclerosis. Other risk factors are glaucoma, syphilis, sarcoidosis, vasculitis, increased intraorbital or intraocular pressure, hyphema, hyperviscosity syndromes (multiple myeloma, Waldenstrom's macroglobulinemia, and leukemia), high homocysteine levels, sickle cell, and HIV .
Paul O'Mahoney et al. studied the relationship between traditional atherosclerosis risk factors and retinal vein occlusion (RVO). They systematically retrieved all studies between 1985 and 2007 that compared cases with any RVO with controls. They concluded that hypertension and hyperlipidemia are common risk factors for RVO in adults, and diabetes mellitus is less so. It remains to be determined whether lowering blood pressure and/or serum lipids levels can improve visual acuity or the complications of RVO .
Every eye with CRVO is at risk for developing neovascular glaucoma. Lowering intraocular pressure helps to improve retinal circulation in an eye with CRVO , and there is a 10% risk for development of BRVO or CRVO in the fellow eye .
Risk factors for developing neovascular iris in patients with CRVO are the amount of nonperfused retina, extent of retinal hemorrhages, male sex, and central vein occlusion of less than one month duration . Visual acuity in patients with CRVO at baseline is a strong predictor for the development of INV/ANV, as is the amount of nonperfusion seen by fluorescein angiogram .
The central vein occlusion study (CVOS) data did not support the recommendation for prophylactic panretinal photocoagulation (PRP). The CVOS found that early PRP decreased the rate of iris neovascularization (INV); however, the reduction was not statistically significant. Moreover, the study showed that early PRP reduced, but did not eliminate, the possibility of anterior-segment neovascularization. The CVOS recommended close follow-up of eyes with CRVO during the first 6 months (including gonioscopy and undilated slit lamp examination of the iris) and prompt PRP of eyes in which iris neovascularization (INV)/Angle neovascularization (ANV) develops .
Although PRP was better than selective PRP or photodynamic therapy (PDT) at determining INV and anterior segment neovascularization regression, selective PRP or PDT can also be safely used to manage anterior segment neovascularization secondary to ischemic CRVO .
Our case illustrates an interesting presentation of unilateral ischemic central retinal vein occlusion, where hypertension and glaucoma were thought to be the main risk factors. While many interventions for fixed visual loss associated with CRVO have largely not proven to be of benefit, our management focused on controlling blood pressure and glaucoma as preventive measures to protect the fellow eye. Meanwhile, close follow up was emphasized to investigate for signs of iris/angle neovascularization.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Central retinal vein occlusion
Central vein occlusion study
- The Central Vein Occlusion Study Group: Natural history and clinical management of central retinal vein occlusion. Arch Ophthalmol. 1997, 115: 486-491.View ArticleGoogle Scholar
- Mohamed Q, McIntosh RL, Saw SM, Wong , TY : Interventions for central retinal vein occlusion: an evidence-based systematic review. Ophthalmology. 2007, 114: 507-10.1016/j.ophtha.2006.11.011.View ArticlePubMedGoogle Scholar
- Klein R, Moss SE, Meuer SM, Klein BE: The 15-year cumulative incidence of retinal vein occlusion: the Beaver Dam Eye Study. Arch Ophthalmol. 2008, 126: 513-10.1001/archopht.126.4.513.View ArticlePubMedGoogle Scholar
- Prisco D, Marcucci R: Retinal vein thrombosis: risk factors, pathogenesis and therapeutic approach. Pathophysiol Haemost Thromb. 2002, 32: 308-10.1159/000073587.View ArticlePubMedGoogle Scholar
- Cugati S, Wang JJ, Knudtson MD, et al: Retinal vein occlusion and vascular mortality: pooled data analysis of 2 population-based cohorts. Ophthalmology. 2007, 114: 520-10.1016/j.ophtha.2006.06.061.View ArticlePubMedGoogle Scholar
- Biousse V, Newman NJ, Sternberg P: Retinal vein occlusion and transient monocular visual loss associated with hyperhomocystinemia. Am J Ophthalmol. 1997, 124: 257.View ArticlePubMedGoogle Scholar
- Freidman N, Kaiser P, Trattler W: Central retinal vein occlusion. Review of ophthalmology. 2005, 329.Google Scholar
- Bhagat N, Goldberg MF, et al: Central retinal vein occlusion: review of management. Eur J Ophthalmol. 1999, 9: 165-180.PubMedGoogle Scholar
- O'Mahoney Paul RA, David T. Wang, et al: Retinal vein occlusion and traditional risk factors for atherosclerosis. Arch Ophthalmol. 2008, 126: 692-699. 10.1001/archopht.126.5.692.View ArticlePubMedGoogle Scholar
- Rath EZ, Frank RN, Shin DH, Kim C: Risk factors for retinal vein occlusions. A case-Control Study Group. Ophthalmology. 1992, 99: 509-514.View ArticlePubMedGoogle Scholar
- Anon: Risk factors for central retinal vein occlusion. The eye disease Case-Control Study Group. Arch Ophthalmol. 1996, 114: 545-554.View ArticleGoogle Scholar
- Hayreh SS: Prevalent misconceptions about acute retinal vascular occlusive disorders. Prog Retin Eye Res. 2005, 24: 493-519. 10.1016/j.preteyeres.2004.12.001.View ArticlePubMedGoogle Scholar
- Central Vein Occlusion Study Group: Baseline and early natural history report. The Central Vein Occlusion Study. Arch Ophthalmol. 1993, 111: 1087.View ArticleGoogle Scholar
- The Central Vein Occlusion Study Group: A randomized clinical trial of early panretinal photocoagulation for ischemic central vein occlusion. The central vein occlusion study group N report. Ophthalmology. 1995, 102: 1434-1444.View ArticleGoogle Scholar
- Porodi , Maurizio Battaglia, et al: Panretinal photocoagulation and photodynamic therapy for anterior segment neovascularization secondary to ischemic central retinal vein occlusion. Ophthalmic surgery, Laser and Imaging. 2007, 38: 94-99.Google Scholar
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