CENTRAL RETINAL ARTERY OCCLUSION

Central retinal artery occlusion (CRAO) occurs in older patients as a complication of atherosclerosis. Rarely, CRAO in the elderly may be related to giant cell arteritis. When CRAO occurs in younger patients, other etiologies such as coagulopathy, cardiac emboli, or collagen vascular disease with vasculitis must be considered.

Symptoms

Pathology/Pathogenesis

Occlusion of the central retinal artery by embolism or thrombosis results in ischemic retinal whitening secondary to infarction of the inner retinal layers and

denaturation of intracellular proteins. Irreversible damage to the neurosensory retina occurs after 90 minutes of complete CRAO.

Sudden, severe, painless loss of vision characterizes the onset of CRAO. Episodes of transient visual loss lasting minutes (amaurosis fugax) may precede permanent visual loss. If eye pain is associated with CRAO, unusual causes such as carotid dissection or orbital cellulitis must be considered. Symptoms of giant cell arteritis including jaw claudication, scalp tenderness, headache, fatigue, and myalgias may occur in some patients.

Clinical Findings

In the majority of eyes with CRAO, visual acuity is 20/200 or worse. Retention of good central acuity may be seen in individuals with a cilioretinal artery. An afferent pupillary defect is invariably present. The characteristic funduscopic lesion in CRAO is a cherry red spot. The cherry red spot refers to the macular appearance of a central red spot surrounded by superficial retinal whitening. Occlusion of the central retinal artery results in ischemia and infarction of the inner retina, including the nerve fiber and ganglion cell layers. This occlusion is manifest by whitening and edema of the inner retina in the macular area where the nerve fiber and ganglion cell layers are thickest. The foveola retains its reddish color because the inner retinal layers are displaced laterally and the underlying choroidal circulation remains intact. Blood flow within retinal arterioles may appear slow and segmented, a finding referred to as “box-car” or “cattle-truck” formation. Iris neovascularization may

be seen in up to 20% of patients with CRAO.

Ancillary Testing

Fluorescein angiography may demonstrate delay or absence of filling in the retinal arteries. Retrograde filling of retinal veins or arteries may be noted. Retinal blood flow may be restored by the time the patient seeks attention, in which case fluorescein angiographic findings may appear normal.

Treatment/Prognosis

Central retinal artery occlusion typically results in severe visual loss in the range of 20/200 to light perception. For embolic CRAO of less than 24 hours’ duration, the treating physician may attempt to improve perfusion pressure and/or “dislodge” the embolus. This may be accomplished by lowering the intraocular pressure (anterior chamber paracentesis, ocular massage, or administration of aqueous suppressants) or dilation of retinal arterioles (having the patient rebreathe into a paper bag or in-

hale carbogen). Treatment rarely alters the visual outcome. Systemic anticoagulation therapy and the use of fibrinolytic agents (given systemically or by selective catherization of the ophthalmic or supraorbital artery) have been tried with variable success. Systemic corticosteroids may be appropriate for CRAO related to vasculitis to protect the fellow eye and limit systemic complications. Prompt panretinal photocoagulation is indicated for eyes that develop iris or anterior chamber angle neovascularization.

Systemic Evaluation

In older patients, the major risk factors for CRAO include hypertension (up to two thirds of patients in one series), diabetes mellitus, carotid artery disease, and coronary artery disease. In patients with atherosclerotic risk factors or evidence of retinal embolization, carotid Doppler studies and echocardiography (either transthoracic or transesophageal) are indicated. If systemic symptoms of vasculitis or collagen vascular disease are present, a sedimentation rate and urgent rheumatological consultation are indicated.

Younger patients require the above embolic workup, with additional consideration given to coagulopathies (sickle cell disease, oral contraceptive use, pregnancy, protein S or antithrombin III deficiency, antiphospholipid antibody syndrome, or homocysteinuria).

An acute embolic central retinal artery occlusion produces sudden vision loss in a 68-year-old man. Visual acuity was 20/400. A prominent cherry red spot was present.

Acute central retinal artery occlusion. Ischemic retinal whitening is present throughout the macula.

A very small cherry red spot is visible.

Acute central retinal artery occlusion with cilioretinal artery sparing is seen in this fundus photograph. Diffuse ischemic retinal whitening of the inner retina is observed. The inferior macula is spared as a result of the cilioretinal artery.

The fluorescein angiogram of the same patient demonstrates nonperfusion of the major retinal arterioles. Only the optic disc and peripapillary vessels fill with fluorescein. The normal choroidal fluorescence is present.

CENTRAL RETINAL VEIN OCCLUSION

Central retinal vein occlusion (CRVO) is a common retinal vascular disease that is usually seen in adults over age 50 years. Men may be more commonly affected than women, especially in younger age groups. Risk factors for CRVO include systemic vascular disease (hypertension, cardiovascular disease, diabetes mellitus) and primary open-angle glaucoma. Central retinal vein occlusions are classified as perfused or nonperfused, based on the extent of retinal ischemia.

Symptoms

Individuals with CRVO may be asymptomatic or present with profound visual loss. Loss of vision may be sudden or gradual. Other symptoms include central scotoma, photopsias, transient visual obscurations, pain, and

eye redness.

Clinical Features

Visual acuity of less than 20/200 and the presence of an afferent pupillary defect suggest ischemia. It is also important to measure intraocular pressure and perform an undilated slit lamp examination and gonioscopy to examine for iris or anterior chamber angle neovascularization. Acute retinal findings include dilated and tortuous veins, scattered intraretinal hemorrhages in all four quadrants of the retina, cotton wool spots, macular edema, and optic disc edema. Late findings include collateral vessels on the optic disc, persistent venous dilation and tortuosity, perivascular sheathing, arterial narrowing, and macular abnormalities (chronic macular edema and retinal pigment epithelial alterations).

The major complications of CRVO are macular edema and neovascular glaucoma. Macular edema may range from mild retinal thickening to frank cystoid macular edema. Approximately one third of eyes with CRVO are classified as nonperfused or ischemic. Of those ischemic eyes, one third to one half will develop neovascular glaucoma as a result of iris and anterior chamber angle neovascularization.

Ancillary Testing

Fluorescein angiography demonstrates a marked delay in the filling of the retinal veins with prolongation of the arteriovenous transit time. Leakage is routinely observed from the optic disc and retinal vessels. Varying degrees of macular ischemia and leakage occur. Central retinal vein occlusions also vary in the extent of peripheral retinal nonperfusion. Eyes with areas of at least 10 disc diameters (DD) of retinal nonperfusion are classified as nonperfused or ischemic.

In addition to fluorescein angiography, the extent of retinal ischemia can be estimated by electrophysiologic testing. Inner retinal ischemia is associated with a reduction in the b-wave amplitude on the electroretinogram. A b-wave to a-wave ratio of less than 1 is associated with significant retinal ischemia and an increased risk for neovascular glaucoma.

Pathology/Pathogenesis

Histopathologic study of autopsy eyes with CRVO reveals thrombosis of the central retinal vein at the level of the lamina cribrosa. Obstruction of the central retinal vein leads to varying degrees of fluid and lipid exudation, red cell extravasation, and ischemia in all quadrants of the retina.

Treatment/Prognosis

The CRVO Study examined the natural history of perfused CRVOs, the effect of macular grid laser photocoagulation for macular edema, and the timing of panretinal photocoagulation (PRP) in eyes with significant nonperfusion. Approximately one third of eyes initially judged as perfused progressed to the nonperfused group by 3 years (most rapidly within the first 4 months). These results indicated that even those patients with initially perfused CRVOs require close follow-up and careful examination for neovascularization of the iris or anterior chamber angle at each visit. Macular gridpattern photocoagulation reduced the extent of macular edema but did not result in significant visual improvement. Prophylactic PRP in eyes with significant retinal ischemia offered no advantage over prompt PRP when iris or angle neovascularization was detected. The CRVO Study recommended patients be examined monthly for the first 6 months. Examination should include undilated slit lamp examination and gonioscopy to detect iris or anterior chamber angle neovascularization. If iris or angle neovascularization is found, PRP should be initiated promptly.

Systemic Evaluation

Common systemic associations with CRVO include hypertension, cardiovascular disease, and diabetes mellitus. Evaluation for a hyperviscosity syndrome is indicated for patients with bilateral simultaneous CRVO. Review of systems in younger patients with CRVO may direct systemic evaluation for hypertension, diabetes, or hypercoagulable states.

Acute central retinal vein occlusion produces optic disc edema and hemorrhage, venous dilation and tortuosity, intraretinal hemorrhages in all four quadrants, and varying degrees of macular edema.

Fluorescein angiogram of an ischemic central retinal vein occlusion reveals widespread retinal capillary nonperfusion. The patient had 20/400 vision and a relative afferent pupillary defect.

Acute central retinal vein occlusion at a higher magnification demonstrates diffuse dot/blot hemorrhages in the midperipheral retina.

Fluorescein angiogram demonstrates chronic macular edema in central retinal vein occlusion. Cystic macular edema is seen with diffuse vascular leakage throughout the macula.

Collateral vessels on the optic disc become apparent within months after the onset of the central retinal vein occlusion. Also note the macular edema associated with lipid exudation.

Iris neovascularization results from widespread retinal ischemia and requires prompt panretinal photocoagulation.

COATS’ DISEASE

Coats’ disease is an idiopathic, developmental retinal vascular abnormality usually affecting one eye of male patients. No genetic predisposition is known. Coats’ disease primarily affects children and young adults; the majority of cases present prior to age 20 years.

Symptoms

The degree of visual loss is variable and usually related to the degree of macular involvement. Infants and children commonly have a more severe variant of the disease and often present with severe visual loss. At the other end of the spectrum, Coats’ disease may be identified on a routine examination in an asymptomatic patient.

Clinical Features

Infants and young children may present with a severe exudative detachment as a result of leakage from telangiectatic changes and aneurysmal dilations of most or all of the retinal vessels. These eyes are at great risk for developing neovascular glaucoma and phthisis bulbi.

A less severe variant, usually occurring in young to middle-aged adults, can present with either macular or peripheral telangiectasia and aneurysmal dilation with varying degrees of leakage and exudation. Juxtafoveal telangiectasia can result in cystoid macular edema and/or macular exudation. Visual loss is usually mild to moderate. Macular telangiectasia may be isolated or associated with peripheral telangiectasia. Peripheral telangiectasia and aneurysmal dilation may also occur as an isolated finding. In such a case, visual loss is due to gravitational deposition of exudate into the macula from the peripheral retinal telangiectasia. The telangiectatic vessels may be associated with retinal capillary nonperfusion and vascular sheathing.

Ancillary Testing

Fluorescein angiography is important in confirming the diagnosis of Coats’ disease. It clearly demonstrates the aneurysmal dilation of the capillary bed, the retinal arteries, and veins. Retinal capillary nonperfusion is often present and more prominent in patients with large aneurysmal dilations. The later phases of the angiogram demonstrate leakage from the telangiectatic vessels.

Pathology/Pathogenesis

The pathogenesis of Coats’ disease is unknown. Histopathologic information derived from eyes enucleated with severe variants of the disease demonstrates irregular dilation of capillaries, arteries, and veins with leakage of periodic acid Schiff (PAS)-positive exudate into the retina and subretinal space. Lipid-engorged macrophages are present in the subretinal space and usually seen remote from the telangiectatic vessels. Electron microscopy demonstrates retinal vascular endothelial abnormalities.

Treatment/Prognosis

Treatment varies according to the severity of disease. Asymptomatic patients with either juxtafoveal or peripheral telangiectasia may not require treatment. These patients should be followed up periodically for progression and instructed to report any changes in vision due to retinal thickening or macular exudation. When vision is threatened or affected, treatment is indicated. Photocoagulation and/or cryotherapy are used to destroy the telangiectatic vessels. Multiple treatment sessions may be necessary. When extensive exudative retinal detachment is present, surgery to drain the subretinal fluid prior to destruction of the telangiectatic vessels may be necessary. A temporary increase in exudation may occur shortly after treatment. If it involves the macula, it may be associated with further loss of vision. Macular distortion secondary to epiretinal membrane formation may follow treatment. The visual prognosis is variable and relates to the degree of macular involvement. Extensive macular lipid deposition may result in permanent changes in the retina and retinal pigment epithelium.

Systemic Evaluation

A systemic workup is usually not indicated in patients presenting with features typical of Coats’ disease. Coats’ disease has been associated with several systemic diseases, including Alport’s disease, tuberous sclerosis, Turner’s syndrome, and Senior-Loken syndrome, as well as muscular dystrophy and fascioscapulohumeral dystrophy. Coats’ disease has also been reported in association with retinitis pigmentosa.

Fundus photograph demonstrates a circinate ring of lipid surrounding the fovea from macular telangiectasia in a 16-year-old boy with a limited variant

of Coats’ disease.

Peripheral retinal telangiectasia with associated intraand subretinal lipid and hemorrhage simulating a peripheral retinal mass in a 12-year-old boy

with Coats’ disease.

Venous-phase fluorescein angiogram of the same patient demonstrates retinal telangiectasia inferior and temporal to the fovea.

Fluorescein angiogram of the same patient demonstrates leaking telangiectatic vessels. Note the associated retinal capillary nonperfusion adjacent to retinal telangiectasia.

A 17-year-old female patient presented with visual loss related to marked submacular lipid exudation. She had extensive peripheral retinal telangiectasis.

In the same patient peripheral areas of exudative retinal detachment are observed.

Diabetic retinopathy is the most common retinal vascular disease. It is the leading cause of new blindness in adults during the third through sixth decades of life. The risk of diabetic retinopathy is related to many factors, including the duration of diabetes and the level of diabetes control. Additional factors, including uncontrolled hypertension, hyperlipidemia, intravascular fluid overload states, renal disease, anemia, pregnancy, and intraocular surgery may aggravate the risk and severity of diabetic retinopathy. Nonproliferative diabetic retinopathy (NPDR) is the most common form of diabetic retinopathy.

Symptoms

Most persons with NPDR have no or minimal symptoms through the preclinical phase prior to the onset of ophthalmoscopically visible vascular lesions. In fact, patients usually do not complain of vision loss until moderate nonproliferative retinopathy develops with the onset of macular edema or ischemia.

Clinical Features

In the preclinical phase, the standard clinical evaluations of ophthalmoscopy and fluorescein angiography are normal. However, patients may have impaired retinal function as indicated by electroretinography, contrast sensitivity, or color vision testing. Nonproliferative diabetic retinopathy is characterized by the presence of microaneurysms, intraretinal hemorrhages, lipid exudate, and cotton wool spots. As the condition progresses, most patients have increased vasodilation and tortuosity. The retinal circulation normally autoregulates the blood supply to match the metabolic demands, as does the brain. However, with progressive retinopathy the autoregulatory mechanisms are overwhelmed, especially by elevated systemic blood pressure, intravascular fluid overload, or hypoalbuminemia. Then the vessels leak, and edema collects in the macula (macular edema), as noted by cystic spaces, retinal thickening, and lipoprotein (“hard” exudate) deposits.

Macular edema is associated with most cases of visual loss in NPDR. The term clinically significant macular edema (CSME) is used to describe those eyes at risk for visual loss related to macular edema. Clinically significant macular edema is defined as any of the following: retinal thickening at or within 500 µm of the center of the macula, lipid exudates at or within 500 µm of the center of

the macula associated with adjacent retinal thickening, and retinal thickening greater than 1 disc diameter (DD) in area within 1 DD of the center of the macula.

The severity of NPDR can be estimated by using the 4-2-1 rule. Eyes with severe NPDR have any one of the following features: 4 quadrants of dot/blot hemorrhage, 2 quadrants of venous beading, or 1 quadrant of intraretinal microvascular abnormality (IRMA).

Ancillary Testing

Fluorescein angiography is performed to determine the degree of macular perfusion and identify the location and extent of treatable lesions in patients with clinically significant macular edema. Treatable lesions include discrete points of retinal hyperfluorescence or leakage (microaneurysms); areas of diffuse leakage (microaneurysms, IRMA, and capillary bed leakage); and areas of retinal capillary nonperfusion (except for the normal foveal avascular zone).

Pathology/Pathogenesis

The clinical features of diabetic retinopathy result from a combination of ocular and systemic factors. The retinal features derive from damage to retinal glial cells, neurons, and retinal vascular cells. For example, the factors that contribute to vascular leakage (such as vascular endothelial growth factor) arise from neurons and glial cells. Loss of vision results from direct or indirect insults to neurons. In addition, systemic factors such as hypertension or fluid overload increase the hydrostatic pressure and aggravate the tendency for the blood vessels to leak.

Treatment/Prognosis

The physiologic features described above serve as principles of therapy. First, the primary systemic metabolic control must be optimized. The Diabetes Control and Complications Trial (DCCT) confirmed the benefit of intensive blood glucose control in reducing the development and progression of diabetic retinopathy in individuals with type 1 diabetes mellitus. Similar results have been demonstrated for those with type 2 diabetes. Second, other cardiovascular risk factors (hypertension, fluid overload, hyperlipidemia, anemia) must be treated. Third, local ocular processes of vascular leakage are treated by focal laser photocoagulation. In eyes with CSME, the Early Treatment Diabetic Retinopathy Study demonstrated that macular laser photocoagulation reduced the risk of moderate visual loss by greater than 50%. Macular photocoagulation for CSME involves both focal laser treatment for leaking microaneurysms and grid-pattern laser photocoagulation for areas of diffuse macular edema.

Systemic Evaluation

The development and progression of diabetic retinopathy is influenced by many factors. Patients with diabetes should undergo regular examination and treatment by their primary care provider or endocrinologist to optimize control of their diabetes and improve their overall medical status.

Clinical findings of nonproliferative diabetic retinopathy include microaneurysms, intraretinal hemorrhages, and lipid exudate. Many patients in the early stages of diabetic retinopathy are asymptomatic.

As diabetic retinopathy progresses, patients develop vasodilation and tortuosity of the retinal vessels. Venous beading is characterized by an irregular dilation of the retinal veins.

Cotton wool spots are common in patients with diabetic retinopathy. They result from microinfarctions in the nerve fiber layer.

The most common cause of visual loss in patients with nonproliferative diabetic retinopathy is macular edema. Macular edema results from retinal vascular leakage and ischemia.

The development and progression of diabetic retinopathy are influenced by many factors including duration of diabetes, quality of diabetes control, and coexisting medical problems. This patient had exacerbation of her diabetic retinopathy related to hypertension.

Hyperlipidemia is a common finding in patients with diabetes. Hyperlipidemia may be associated with a significant increase in the presence of retinal lipid exudate and visual loss, as seen in this patient.

Clinically significant macular edema is determined by the location and extent of the edema. This patient had retinal thickening and lipid exudate involving the center of the macula.

The same patient also had retinal thickening greater than 1 disc diameter in size. Her fluorescein angiogram revealed numerous microaneurysms in the temporal macula.

Clinically significant macular edema (CSME) is a clinical diagnosis based on careful fundus contact lens ophthalmoscopic examination. This patient had CSME with retinal thickening and lipid exudate within 500 µm of the center of the macula.

A fluorescein angiogram of the same patient was obtained to identify treatable lesions before laser photocoagulation. The numerous hyperfluorescent spots are consistent with microaneurysms.

The fluorescein angiogram of the same patient reveals enlargement of the foveal avascular zone, along with areas of capillary nonperfusion.

Throughout the study, fluorescein leaks from the microaneurysms resulting in increased hyperfluorescence.

Macular edema results not only from vascular leakage but also from retinal ischemia. This 64-year-old man had clinically significant macular edema with diffuse retinal thickening in his right eye.

The severity of diabetic retinopathy can be estimated using the 4-2-1 rule. Eyes with severe nonproliferative diabetic retinopathy have any one of the following features: 4 quadrants of dot/blot hemorrhage, 2 quadrants of venous beading, or 1 quadrant of intraretinal microvascular abnormalities.

Fluorescein angiography of the same patient reveals hypofluorescence throughout the macula related to capillary nonperfusion.

Patients with diabetic retinopathy rarely may develop a pseudovasculitis. The perivascular sheathing is likely the result of lipid exudate along the vessel wall.

Diabetic papillopathy is a cause of temporary visual loss in patients with type 1 diabetes. Patients present with sudden visual loss in one eye. The optic disc is swollen and hyperemic with superficial optic disc and retinal hemorrhages.

The disc edema and hemorrhages in the same patient resolved over weeks to months. Visual acuity returned to normal.

Diabetic retinopathy is the most common retinal vascular disease. It is the leading cause of new blindness in adults during the third through sixth decades of life. Proliferative diabetic retinopathy (PDR) is an advanced form of diabetic retinopathy characterized by the proliferation of new vessels on the optic disc, retina, or iris as

a result of widespread retinal ischemia. Complications of PDR include vitreous hemorrhage, fibrovascular proliferation, traction retinal detachment, and neovascular glaucoma.

Symptoms

Individuals with PDR may be asymptomatic or complain of visual loss and floaters related to vitreous hemorrhage. In cases of neovascular glaucoma, patients may present with eye pain and redness in addition to loss of vision.

Clinical Features

Individuals with PDR exhibit the same findings as patients with nonproliferative diabetic retinopathy: microaneurysms, retinal hemorrhages, cotton wool spots, lipid exudates, and macular edema. In addition, neovascularization arises on the optic disc, retina, and/or iris. Neovascularization of the optic disc (NVD) appears as fine, lacy vessels on the surface of the optic disc. Neovascularization elsewhere (NVE) is retinal neovascularization located at sites other than on or within 1 disc diameter (DD) of the optic disc. Neovascularization elsewhere is observed most commonly along or just anterior to the temporal retinal vascular arcades. Neovascularization of the optic disc and neovacularization elsewhere are associated with a variable amount of fibrosis.

The vitreous plays a critical role in the development of PDR. It appears that the vitreous provides a scaffold for the growth of new vessels. The strong interaction between the vitreous and the neovascular tissue contributes to the development of vitreous hemorrhage and traction retinal detachment.

Iris neovascularization is characterized by the proliferation of fine, lacy vessels on the iris surface or anterior chamber angle. Frank neovascular glaucoma may present with conjunctival injection, corneal edema, anterior chamber flare, iris and angle neovascularization, and intraocular pressure elevation.

Ancillary Testing

Fluorescein angiography reveals significant midperipheral capillary nonperfusion. Neovascularization of

the optic disc or neovascularization elsewhere hyperfluoresces early and leaks throughout the study. Neovascularization elsewhere usually is observed at the junction of the perfused and nonperfused retina.

Pathology/Pathogenesis

The risk of PDR is related to the extent of retinal ischemia. Vascular endothelial growth factor (VEGF) is a leading candidate linking retinal ischemia and intraocular neovascularization. This growth factor is an angiogenic peptide, the expression of which is markedly increased by retinal hypoxia. Conversely, VEGF

levels decline significantly after successful panretinal photocoagulation (PRP).

Treatment/Prognosis

The Diabetic Retinopathy Study identified four retinopathy risk factors for severe visual loss: new vessels present, NVD, preretinal or vitreous hemorrhage, and severe new vessels (NVD greater than one-third disc area, or in the absence of NVD, NVE greater than one-half DD). The presence of three or four risk factors indicates “high risk” for severe visual loss and requires prompt PRP. Panretinal photocoagulation is successful in reducing the risk of severe visual loss by more than 50%. A favorable response to PRP is associated with a regression of retinopathy risk factors, which in turn is associated with a favorable visual prognosis. The beneficial effects of PRP are long standing. In eyes with nonclearing vitreous hemorrhage or traction retinal detachment, pars plana vitrectomy may be performed. Iris neovascularization and neovascular glaucoma are treated with prompt PRP.

Systemic Evaluation

The development and progression of diabetic retinopathy are influenced by many factors. Patients with diabetes should undergo regular examination and treatment by their primary care provider or endocrinologist to optimize their diabetes control and improve their overall medical status. Particular attention should be directed to the treatment of hypertension and renal failure or other fluid overload states.

Optic disc neovascularization (NVD) is characterized by the growth of fine, lacy vessels from the optic disc. If NVD is larger than one-third disc area in size, it is classified as high-risk proliferative diabetic retinopathy.

Neovascularization elsewhere is found along the major vascular arcades or in the midperipheral retina.

Patients with neovascularization of the optic disc may be asymptomatic early in the course of proliferative diabetic retinopathy. Visual loss is related to the development of vitreous hemorrhage or traction retinal detachment.

Fibrovascular proliferation causes areas of adhesion between the optic disc or retina and the posterior vitreous. Contraction of the fibrovascular tissue results in vitreous hemorrhage or traction retinal detachment.

Iris and angle neovascularization is a severe complication of proliferative diabetic retinopathy. Secondary closure of the anterior chamber angle leads to neovascular glaucoma. Prompt panretinal photocoagulation is required for patients with iris or angle neovascularization.

The development of proliferative diabetic retinopathy is a result of widespread retinal ischemia. This fluorescein angiogram demonstrates peripheral hypofluorescence as a result of retinal capillary nonperfusion.

Neovascularization elsewhere is found most commonly along the temporal vascular arcades.

The new vessels are located at the junction of the perfused and nonperfused retina. The abnormal vessels hyperfluoresce intensely with fluorescein angiography.

Preretinal hemorrhage may assume a boat-shaped pattern. This patient also had hyperlipidemia with extensive lipid exudation.

The hemorrhage obscures the macula and retinal blood vessels, confirming the preretinal location. This preretinal hemorrhage is associated with a smoky rim of fibrin along the superior margin.

Fluorescein angiogram of a 24-year-old woman with proliferative diabetic retinopathy. Extensive peripheral capillary nonperfusion is evident.

The areas of intense hyperfluorescence on the optic disc and along the vascular arcades are suggestive of neovascularization.

Untreated proliferative diabetic retinopathy may result in extensive fibrovascular proliferation, as shown in this patient. Fibrovascular proliferation may lead to vitreous hemorrhage and traction retinal detachment.

Panretinal photocoagulation (PRP) is indicated for the treatment of high-risk proliferative diabetic retinopathy. This fundus photograph was taken immediately after PRP. The white burns are visible inferior to the preretinal, boat-shaped hemorrhage.

Patients with traction retinal detachments may occasionally develop retinal holes and secondary rhegmatogenous retinal detachments. The white area is the point of vitreoretinal traction.

Fundus photograph of a patient successfully treated with panretinal photocoagulation approximately 6 months previously for high-risk proliferative diabetic retinopathy.

This 24-year-old woman had high-risk proliferative diabetic retinopathy that did not respond to standard panretinal photocoagulation. She developed extensive fibrovascular proliferation with vitreous hemorrhage.

She did well after pars plana vitrectomy and supplemental laser photocoagulation.

EALES’ DISEASE

Eales’ disease is a primary, idiopathic obliterative vasculopathy that most often affects young adults between 20 and 30 years of age. Men are affected more commonly than women. Eales’ disease is characterized by perivascular sheathing, peripheral retinal nonperfusion, neovascularization, and recurrent vitreous hemorrhages. The disease is bilateral but may be asymmetric.

Symptoms

Most patients present with symptoms related to recurrent vitreous hemorrhage, including floaters and reduced vision. Others may complain of blurred vision not associated with floaters.

Clinical Features

Patients with Eales’ disease typically have evidence of retinal venous and/or arterial sheathing, telangiectatic changes in the midperipheral retina, and optic disc or peripheral retinal neovascularization. Preretinal or vitreous hemorrhage is common. Fibrovascular proliferation may lead to traction retinal detachment. Macular abnormalities include cystoid macular edema and epiretinal membrane formation. Some patients have vitreous cells but anterior segment inflammation is uncommon.

The major differential diagnostic considerations include other causes of peripheral retinal neovascularization such as peripheral branch retinal vein occlusion, sickle cell retinopathy, multiple sclerosis, and sarcoidosis.

Ancillary Testing

Fluorescein angiography shows midperipheral retinal nonperfusion with well-demarcated borders between areas of perfused and nonperfused retina. Leakage of fluorescein from areas of neovascularization may be observed from the optic disc or located at the junction of perfused and nonperfused retina.

Pathology/Pathogenesis

Little is understood of the pathogenesis. Peripheral retinal inflammation may incite an obliterative vasculitis leading to subsequent ischemia and neovascularization.

Treatment/Prognosis

Laser photocoagulation to the ischemic peripheral retina is the treatment of choice, but cryotherapy may used if the vitreous is opaque. The inflammatory component and macular edema may respond to periocular or systemic corticosteroids. Most patients have a good prognosis, but rare patients develop macular ischemia or traction retinal detachment.

Systemic Evaluation

Most patients diagnosed with Eales’ disease in the United States are healthy and without associated systemic disease. However, there is an association with tuberculosis, especially in the Middle East, so tuberculin skin testing is recommended.

This 31-year-old man with Eales’ disease had a history of intermittent vision loss. He had vitreous opacities consistent with old hemorrhage and areas of vasculitis involving the retinal veins (periphlebitis).

Peripheral retinal examination of the same patient revealed retinal neovascularization with fibrovascular proliferation and traction retinal detachment.