BASICS
DESCRIPTION
An idiopathic obliterative vasculopathy. The classic triad is a retinal phlebitis, associated with peripheral retinal nonperfusion and vitreous hemorrhage, typically in young adults.
EPIDEMIOLOGY
Incidence
• Most commonly reported in India and the Middle East.
• In India, it occurs in 1/250 patients with eye disease.
• It usually occurs in adults 20–30 years old.
• Prior studies suggested a higher incidence in men; however recent reports suggest a more even distribution between men and women.
RISK FACTORS
Living in areas such as India and the Middle East.
PATHOPHYSIOLOGY
A nonspecific obliterative vasculitis.
A hypersensitivity to tuberculin protein has been suggested, and tubercle bacilli have been identified in pathology specimens.
COMMONLY ASSOCIATED CONDITIONS
Myelopathy, ischemic stroke, hemiplegia, multifocal white matter abnormalities, and vestibuloauditory dysfunction have been reported.
DIAGNOSIS
HISTORY
The most common symptoms include decreased vision and cobwebs or floaters.
PHYSICAL EXAM
• Retinal phlebitis appears as vascular sheathing with adjacent nerve fiber layer hemorrhages and hard exudates.
• Over half of patients will have bilateral disease.
• Anterior chamber cell and flare with keratic precipitates may be present. Vitreous debris and cells, as well as vitreous hemorrhage, can be seen.
• Cystoid macular edema may be present.
• Nonperfusion in the temporal periphery is typical and is associated with other microvascular changes such as microaneurysms, venous shunting, venous beading, hard exudates, and cotton wool spots. Neovascularization of the disc or elsewhere in the retina is present in up to 80% of patients. Neovascular glaucoma may also occur.
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
No specific testing can determine the diagnosis; however Eales disease is a diagnosis of exclusion and testing should be done to rule out causes of vasculitis (see Differential Diagnosis section).
Follow-up & special considerations
Lumbar puncture (not routinely needed) shows pleocytosis of CSF. In addition, MRI and CT imaging may be used to identify CNS lesions.
Imaging
Initial approach
• Fluorescein angiography (FA) is helpful to analyze presence of active vasculitis, vascular damage, and to look for areas of nonperfusion and neovascularization.
• OCT may reveal cystoid macular edema and/or epiretinal membranes.
Follow-up & special considerations
Patients should be monitored for neovascularization of the retina, iris, and anterior chamber angle. Retinal neovascularization may lead to vitreous hemorrhage. Epiretinal membranes may develop after pan-retinal photocoagulation (but may also occur without laser photocoagulation).
Diagnostic Procedures/Other
• Eales disease is diagnosis of exclusion and therefore entities in the differential diagnosis must be ruled out with appropriate history, physical exam, and ancillary testing.
• MRI testing of the CNS may reveal multifocal white matter abnormalities.
DIFFERENTIAL DIAGNOSIS
• Sickle cell disease.
• Diabetes mellitus.
• Branch retinal vein or artery occlusion.
• Retinal embolization.
• Retinopathy of prematurity.
• Familial exudative vitreoretinopathy.
• Hyperviscosity syndromes (e.g., leukemia).
• Ocular ischemic syndrome.
• Carotid–cavernous fistula.
• Multiple sclerosis.
• Toxemia of pregnancy.
• Sarcoidosis.
• Collagen-vascular disease.
• Vasculitis secondary to infection.
• Uveitis.
• Birdshot retinochoroidopathy.
• Toxoplasmosis.
• Acute retinal necrosis.
• Long-standing retinal detachment.
• Retinitis pigmentosa.
• Retinoschisis.
• Choroidal melanoma or hemangioma.
• Incontinentia pigmenti.
TREATMENT
ADDITIONAL TREATMENT
Issues for Referral
CNS findings warrant a referral to neurology.
COMPLEMENTARY & ALTERNATIVE THERAPIES
Thyroid extract, osteogenic hormones, androgenic hormones, and systemic steroids and antioxidant vitamins A, C, and E have been suggested. None have been proven effective in the treatment of this disease.
SURGERY/OTHER PROCEDURES
• Panretinal photocoagulation for neovascular complications is necessary.
• Vitrectomy and membrane removal may be considered for epiretinal membranes.
• Both oral steroids and intravitreal triamcinolone has been used to stabilize vascular leakage.
• Intravitreal bevacizumab has been used to induce regression of neovascularization.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
Patients should been followed every 3 to 12 months, depending on the extent of nonperfusion and neovascularization.
PATIENT EDUCATION
Patients should be taught the symptoms of vitreous hemorrhage, such as sudden onset of floaters. Early detection and treatment of vitreous hemorrhage and neovascularization can allow laser photocoagulation, which may prevent the tractional complications such as retinal detachment or neovascular glaucoma.
PROGNOSIS
The majority of patients maintains 20/40 vision or better with disease stabilization.
COMPLICATIONS
Retinal neovascularization, rhegmatogenous retinal detachment, vitreous hemorrhage, and traction retinal detachment can also occur.
REFERENCES
1. Chanana B, Azad RV, Patwardhan S. Role of intravitreal bevacizumab in the management of Eales’ disease. Int Ophthalmol 2010;30(1):57–61. E-pub Jan 23, 2009.
2. Das T, Pathengay A, Hussain N, Biswas J. Eales disease: Diagnosis and management. Eye 2010;24:472–482.
3. Ishaq M, Feroze AH, Shahid M, et al. Intravitreal steroids may facilitate treatment of Eales’ disease (idiopathic retinal vasculitis): An interventional case series. Eye (Lond) 2007;21(11):1403–1405. E-pub Sept 15, 2006.
4. Therese KL, Deepa P, Therese J, Bagyalakshmi R, Biswas J, Madhavan HN. Association of mycobacteria with Eales’ disease. Ind J Med Res 2007;126(1):56–62.
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