Central Retinal Vein Occlusion 

  • Author: Lakshmana M Kooragayala, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 5, 2011
 

Background

Central retinal vein occlusion (CRVO) is a common retinal vascular disorder. Clinically, CRVO presents with variable visual loss; the fundus may show retinal hemorrhages, dilated tortuous retinal veins, cotton-wool spots, macular edema, and optic disc edema. Note the images below.

Recent onset central retinal vein occlusion, showiRecent onset central retinal vein occlusion, showing extensive hemorrhages in the posterior pole and giving the "blood and thunder appearance." Peripheral fundus view of the same patient as in tPeripheral fundus view of the same patient as in the previous image, showing hemorrhages extending all over the fundus. Fluorescein angiograph of same patient as in previFluorescein angiograph of same patient as in previous images, showing hypofluorescence due to blockage from hemorrhages in the retina. It is not useful to perform a fluorescein angiogram in acute stages of the disease. Fundus picture of the same patient as in previous Fundus picture of the same patient as in previous images, showing resolving neovascularization of the disc and panretinal photocoagulation scars. Fluorescein angiogram of the same patient as in thFluorescein angiogram of the same patient as in the previous images, taken more than 1 year later, showing persistent cystoid macular edema with good laser spots.

In view of the devastating complications associated with the severe form of CRVO, a number of classifications were described in the literature. All of these classifications take into account the area of retinal capillary nonperfusion and the development of neovascular complications.[1, 2, 3, 4, 5]

Broadly, CRVO can be divided into 2 clinical types, ischemic and nonischemic. In addition, a number of patients may have an intermediate in presentation with variable clinical course. On initial presentation, it may be difficult to classify a given patient into either category, since CRVO may change with time.

A number of clinical and ancillary investigative factors are taken into account for classifying CRVO, including vision at presentation, presence or absence of relative afferent pupillary defect, extent of retinal hemorrhages, cotton-wool spots, extent of retinal perfusion by fluorescein angiography, and electroretinographic changes.

Nonischemic CRVO is the milder form of the disease. It may present with good vision, few retinal hemorrhages and cotton-wool spots, no relative afferent pupillary defect, and good perfusion to the retina. Nonischemic CRVO may resolve fully with good visual outcome or may progress to the ischemic type. Note the images below.

Patient with nonischemic central retinal vein occlPatient with nonischemic central retinal vein occlusion presented with dilated, tortuous veins and superficial hemorrhages. Fundus picture of the same patient as in previous Fundus picture of the same patient as in previous image, showing resolved hemorrhages and pigmentary changes in the macula several months later.

Ischemic CRVO is the severe form of the disease. CRVO may present initially as the ischemic type, or it may progress from nonischemic. Usually, ischemic CRVO presents with severe visual loss, extensive retinal hemorrhages and cotton-wool spots, presence of relative afferent pupillary defect, poor perfusion to retina, and presence of severe electroretinographic changes. In addition, patients may end up with neovascular glaucoma and a painful blind eye.

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Pathophysiology

The exact pathogenesis of the thrombotic occlusion of the central retinal vein is not known. Various local and systemic factors play a role in the pathological closure of the central retinal vein.[3, 6, 7]

The central retinal artery and vein share a common adventitial sheath as they exit the optic nerve head and pass through a narrow opening in the lamina cribrosa. Because of this narrow entry in the lamina cribrosa, the vessels are in a tight compartment with limited space for displacement. This anatomical position predisposes to thrombus formation in the central retinal vein by various factors, including slowing of the blood stream, changes in the vessel wall, and changes in the blood.

Arteriosclerotic changes in the central retinal artery transform the artery into a rigid structure and impinge upon the pliable central retinal vein, causing hemodynamic disturbances, endothelial damage, and thrombus formation. This mechanism explains the fact that there will be an associated arterial disease with CRVO. However, this association has not been proven consistently, and various authors disagree on this fact.

Thrombotic occlusion of the central retinal vein can occur as a result of various pathologic insults, including compression of the vein (mechanical pressure due to structural changes in lamina cribrosa, eg, glaucomatous cupping, inflammatory swelling in optic nerve, orbital disorders); hemodynamic disturbances (associated with hyperdynamic or sluggish circulation); vessel wall changes (eg, vasculitis); and changes in the blood (eg, deficiency of thrombolytic factors, increase in clotting factors).

Occlusion of the central retinal vein leads to the backup of the blood in the retinal venous system and increased resistance to venous blood flow. This increased resistance causes stagnation of the blood and ischemic damage to the retina. It has been postulated that ischemic damage to the retina stimulates increased production of vascular endothelial growth factor (VEGF) in the vitreous cavity. Increased levels of VEGF stimulate neovascularization of the posterior and anterior segment (responsible for secondary complications due to CRVO). Also, it has been shown that VEGF causes capillary leakage leading to macular edema (which is the leading cause of visual loss in both ischemic CRVO and nonischemic CRVO).

The prognosis of CRVO depends upon the reestablishment of patency of the venous system by recanalization, dissolution of clot, or formation of optociliary shunt vessels.

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Epidemiology

Frequency

United States

CRVO and branch retinal vein occlusion constitute the second most common retinal vascular disorder. The nonischemic type is more common than the ischemic type.

In a recent publication, the Beaver Dam Eye Study Group reported the 15-year cumulative incidence of CRVO to be 0.5%.[8]

International

A large population-based study in Israel reported a 4-year incidence of retinal vein occlusion of 2.14 cases per 1000 of general population older than 40 years and 5.36 cases per 1000 of general population older than 64 years.

In Australia, the prevalence of vein occlusion ranges from 0.7% in patients aged 49-60 years to 4.6% in patients older than 80 years.[9]

Mortality/Morbidity

CRVO is not associated directly with increased mortality.

Nonischemic CRVO may resolve completely without any complications in about 10% of cases. In about 50% of patients, vision may be 20/200 or worse. One third of patients may progress to the ischemic type, commonly in the first 6-12 months after presentation.

In more than 90% of patients with ischemic CRVO, final visual acuity may be 20/200 or worse. Anterior segment neovascularization with associated neovascular glaucoma develops in more than 60% of cases. This can happen within a few weeks and up to 1-2 years afterward.

It has been reported that the fellow eye may develop retinal vein occlusion in about 7% of cases within 2 years. In another report, the 4-year risk of developing second venous occlusion is 2.5% in the same eye and 11.9% in the fellow eye. Neovascular glaucoma may result in a painful blind eye.

Race

CRVO does not have any particular racial preference.

Sex

CRVO occurs slightly more frequently in males than in females.

Age

More than 90% of CRVO occurs in patients older than 50 years, but it has been reported in all age groups.

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Contributor Information and Disclosures
Author

Lakshmana M Kooragayala, MD  Vitreo-retinal Surgeon, Marietta Eye Clinic

Lakshmana M Kooragayala, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, and Medical Association of Georgia

Disclosure: Nothing to disclose.

Specialty Editor Board

V Al Pakalnis, MD, PhD  Professor of Ophthalmology, University of South Carolina School of Medicine; Chief of Ophthalmology, Dorn Veterans Affairs Medical Center

V Al Pakalnis, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and South Carolina Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

References

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Recent onset central retinal vein occlusion, showing extensive hemorrhages in the posterior pole and giving the "blood and thunder appearance."
Peripheral fundus view of the same patient as in the previous image, showing hemorrhages extending all over the fundus.
Fluorescein angiograph of same patient as in previous images, showing hypofluorescence due to blockage from hemorrhages in the retina. It is not useful to perform a fluorescein angiogram in acute stages of the disease.
Fundus picture of the same patient as in previous images, showing resolving neovascularization of the disc and panretinal photocoagulation scars.
Fluorescein angiogram of the same patient as in the previous images, taken more than 1 year later, showing persistent cystoid macular edema with good laser spots.
Patient with nonischemic central retinal vein occlusion presented with dilated, tortuous veins and superficial hemorrhages.
Fundus picture of the same patient as in previous image, showing resolved hemorrhages and pigmentary changes in the macula several months later.
Central retinal vein occlusion showing significant disc edema with dilated tortuous veins and scattered retinal hemorrhages.
Fluorescein angiogram of the same patient in as in previous image, showing leakage from disc, staining of retinal veins.
Fundus of a patient with nonischemic central retinal vein occlusion, showing few scattered peripheral fundus hemorrhages.
Scattered retinal hemorrhages in a patient with central retinal vein occlusion.
Fluorescein angiogram of a patient with nonischemic central retinal vein occlusion, showing staining of dilated tortuous veins with leakage into macula in a cystoid pattern.
Fluorescein angiogram of the same patient as in previous image, showing perifoveal capillary leakage in a cystoid pattern in late phases of angiogram.
Late phase of fluorescein angiograph of the same patient as in previous image, showing cystoid pattern of leakage from perifoveal dilated leaking capillary network.
Arteriovenous phase of fluorescein angiograph showing perifoveal capillary leakage in a patient with nonischemic central retinal vein occlusion.
Fundus picture of a well-compensated, old central retinal vein occlusion showing optociliary shunt vessels.
Red-free photo of the same patient as in the previous image, showing prominent optociliary shunt vessels.
 
 
 
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