Excerpt from Central Retinal Vein Occlusion

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

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.

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.

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.

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

Arteriosclerotic changes in the central retinal artery transforms the artery into a rigid structure and impinges 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 blood (eg, deficiency of thrombolytic factors, increase in clotting factors).

Occlusion of the central retinal vein leads to the backup of blood in the retinal venous system and increased resistance to venous blood flow. This increased resistance causes stagnation of 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).

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

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.

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, prevalence of vein occlusion ranges from 0.7% in patients aged 49-60 years to 4.6% in patients older than 80 years.

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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