eMedicine Specialties > Ophthalmology > Retina

Eales Disease

Daniel B Roth, MD, Assistant Clinical Professor, Department of Ophthalmology, University of Medicine and Dentistry of New Jersey

Updated: Dec 14, 2007

Introduction

Background

Eales disease is an idiopathic obliterative vasculopathy that usually involves the peripheral retina of young adults. In 1880, Henry Eales first described it in healthy young men with abnormal retinal veins and recurrent vitreal hemorrhages.

Clinical findings are characterized by avascular areas in the retina periphery, followed posteriorly by microaneurysms, dilatation of capillary channels, tortuosity of neighboring vessels, and spontaneous chorioretinal scars. It is a diagnosis of exclusion, as many other retinal disorders can mimic Eales disease, especially conditions of retinal inflammation or neovascularization.

Pathophysiology

The pathophysiology of Eales disease is mostly unknown. It is believed to be a primary, noninflammatory disorder of the walls of peripheral retinal vessels, namely the shunt vessels. This often leads to vascular occlusions, peripheral neovascularization, and vitreous hemorrhage. The microvascular abnormalities are seen at the junction of perfused and nonperfused zones of the retina. Although associations with tuberculosis and multiple sclerosis have been suggested, these findings have not been substantiated in other studies. It is possible that the association of Eales disease with both ocular inflammation and sensitivity to tuberculin protein suggests that this disease may be associated with immunologic phenomena whose mechanisms remain unknown.

Frequency

United States

Eales disease is uncommon, with most reports based upon a series of cases.

International

Eales disease is most commonly seen in India and portions of the Middle East.

Mortality/Morbidity

No known mortality is associated with Eales disease. Visual compromise is seen in patients with recurrent vitreous hemorrhage; however, the visual acuity resolves to better than 20/200 in greater than 70% of patients. If retinal nonperfusion extends into the macula, the visual acuity usually is worse than 20/400.

Race

No racial predilection is known in Eales disease; however, the disease is more prevalent in India and portions of the Middle East.

Sex

Young adult males have been reported to have an increased prevalence; however, a study of 55 patients by Gieser and Murphy found that men and women are affected equally.¹

Age

Peak age of onset is 20-35 years, with a reported range of 13-63 years.

Clinical

History

Most patients present with symptoms of floaters, specks, cobwebs, blurring, or decreased vision associated with vitreous hemorrhage. Other patients have blurring associated with retinal vasculitis or uveitis, but without hemorrhage. Often, patients complain of uniocular symptoms, but ophthalmic examination reveals early changes of Eales disease in the other eye. Bilateral involvement is evident in 80-90% of patients.

Physical

• The physical findings mostly involve the retina and vitreous. Vascular sheathing with adjacent nerve fiber layer hemorrhages is seen in most patients. The sheathing can manifest as thin white lines, limiting the blood column on both sides of the sheathed vessel to heavy exudative sheathing that can cause vascular occlusion. Although believed to affect primarily the retinal veins, others have reported the same prevalence of both venules and arterioles. Areas of vascular sheathing often leak dye on fluorescein angiography (FA).
• The anterior chamber may exhibit cell and flare with keratic precipitates. Vitreous debris and cells often are seen, even in the absence of vitreous hemorrhage. Macular edema can occur in eyes with vascular sheathing, and it often is cystoid in nature. Epiretinal membranes with or without macular edema can compromise visual acuity. The etiology of the macular edema is thought to be associated with low-grade inflammation.
• Peripheral nonperfusion is a typical feature of Eales disease. The temporal retina is affected most commonly, often in a confluent area. The surrounding vasculature is tortuous with microvascular abnormalities, which include the following: microaneurysms, arteriovenous shunts, venous beading, hard exudates, and cotton-wool spots. Fine solid white lines occasionally can be seen, representing obliterated larger vessels.
• Branch retinal vein occlusion (BRVO) can be seen in patients with Eales disease and may be limited to one area or may be multifocal. BRVO alone can be differentiated from BRVO in the presence of Eales disease by the more extensive peripheral retinal involvement in Eales disease. BRVO alone usually is confined to a single affected quadrant. BRVO alone also respects the anatomical distribution of the horizontal raphe, unlike Eales disease.
• Neovascularization of the disc (NVD) or neovascularization elsewhere (NVE) in the retina is observed in up to 80% of patients with Eales disease. The NVE usually is located peripherally, at the junction of perfused and nonperfused retina. The neovascularization often is the source of vitreous hemorrhage in these eyes, compromising vision. Rubeosis iridis or neovascularization of the iris can develop and may lead to neovascular glaucoma. Fibrovascular proliferation on the surface of the retina may accompany retinal neovascularization. These eyes have associated anteroposterior traction that could lead to retinal detachment.
• Cystoid macular edema can occur in patients with Eales disease due to increased capillary permeability. This can often be associated with significant vision loss.
• A posterior vitreous separation has been reported in 27% of patients with Eales disease, and several patients have been found to have concomitant macular holes. Macular hole surgery may effectively repair this abnormality and lead to significant visual improvement similar to that seen in patients with idiopathic macular holes.
• Systemic abnormalities have been reported in association with Eales disease, mostly neurologic findings. Myelopathy, ischemic stroke, hemiplegia, and multifocal white matter abnormalities have been reported. A higher incidence of vestibuloauditory dysfunction is seen in patients with Eales disease when compared to the general population of the same age. It is presumed that a similar mechanism of vascular occlusion and hypoxia leads to these systemic findings.

Causes

The cause of Eales disease is unknown. It is a diagnosis of exclusion and is thought to be idiopathic. No causative drugs, environmental factors, or infectious agents have been identified. Although a hypersensitivity to tuberculin protein has been reported, no clear relationship to tuberculosis has been found.

Differential Diagnoses

Branch Retinal Vein Occlusion
Central Retinal Vein Occlusion
Retinopathy, Diabetic, Proliferative
Retinopathy, Hemoglobinopathies
Sarcoidosis

Other Problems to Be Considered

Systemic lupus erythematosus
Collagen vascular disease
Idiopathic periphlebitis

Workup

Laboratory Studies

• No clinical laboratory studies are available to confirm the diagnosis of Eales disease, as it is a diagnosis of exclusion. However, laboratory studies are necessary to exclude other similar entities.
  • Blood glucose or fasting blood glucose to rule out diabetes mellitus
  • Complete blood count to rule out hemoglobinopathies
  • Sickle cell preparation and serum protein electrophoresis to rule out sickle cell disease
  • Angiotensin converting enzyme and lysozyme to rule out sarcoidosis
  • Antinuclear antibody, rheumatoid factor, and erythrocyte sedimentation rate to rule out collagen vascular disease
  • Tuberculin skin testing may be useful, as patients with Eales disease have a higher incidence of tuberculin hypersensitivity, despite the absence of a history of tuberculosis.

Imaging Studies

• FA can be useful to determine the nature of the microvascular abnormalities. Neovascularization and exudative sheathing of vessels will leak fluorescein dye. The area and degree of nonperfusion can be determined on FA and help delineate where to apply laser photocoagulation, when indicated.
• Echographic evaluation often is useful to evaluate the retina in the setting of vitreous hemorrhage. When the details of the fundus are obscured, ultrasound can determine the presence or absence of a retinal detachment or vitreoretinal traction.
• A chest x-ray may be considered to rule out sarcoidosis or a history of tuberculosis, in the setting of a positive tuberculin skin test.
• Magnetic resonance imaging (MRI) of the brain may reveal multifocal white matter abnormalities, but this study probably is not warranted in the absence of neurologic symptoms.

Other Tests

• Studies have found an increased level of oxidation and peroxidation products in vitreous samples from patients with Eales disease (ie, an increased amount of thiobarbituric acid reacting substances). A decreased level of antioxidant enzymes also has been found in vitreous samples from patients with Eales disease (ie, a decreased amount of reduced glutathione, superoxide dismutase, and glutathione peroxidase).
• Hearing and balance should be tested formally, as patients with Eales disease may have associated vestibuloauditory dysfunction.

Treatment

Medical Care

• Several treatments have been proposed for Eales disease; however, none of these treatments is of proven benefit. Treatments include thyroid extract, osteogenic hormones, androgenic hormones, and systemic steroids. The antioxidant vitamins A, C, and E have been suggested as a possible therapy because antioxidizing enzymes are deficient in the vitreous samples of patients with Eales disease.
• In cases complicated by cystoid macular edema, intravitreal triamcinolone acetonide has been effectively used in reversing the edema and in leading to visual improvement. Doses of 1-25 mg of triamcinolone have been reported; however, doses of 2-4 mg of triamcinolone are more commonly used in clinical practice.

Surgical Care

• Moderately light, full-scatter laser photocoagulation to areas of nonperfused retina has become the treatment of choice in patients with Eales disease. The junctional area between perfused and nonperfused retina is to be treated. This treatment results in resolution of neovascularization of the disc, elsewhere in the retina, or the iris, and lowers the incidence of vitreous hemorrhage.
• A major cause of visual loss in patients with Eales disease results from recurrent vitreous hemorrhage. Although the hemorrhage often settles in the inferior portion of the vitreous and reabsorbs within several weeks, surgical intervention occasionally is indicated. Pars plana vitrectomy is effective in removing nonclearing vitreous hemorrhage and enabling adequate scatter laser photocoagulation. In cases of tractional retinal detachment, vitrectomy in combination with membrane dissection is necessary.

Consultations

Consultation with a neurologist is appropriate in the setting of concomitant neurologic symptoms.

Diet

No dietary modification has been proven to be of benefit in Eales disease. However, studies suggest that vitamin A, C, and E supplementation may have a beneficial effect.

Activity

No limitations in activity exist for patients with Eales disease. However, in the setting of vitreous hemorrhage, a sitting position may expedite the clearing of the central visual axis, as the blood is reabsorbed.

Medication

No drugs have been found to be effective in the treatment of the underlying mechanism of Eales disease. Medications used in different studies are mentioned above, but they have not been proven to be beneficial. Cystoid macular edema can complicate Eales disease and has responded to periocular and intravitreal triamcinolone.

Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. Modify the body's immune response to diverse stimuli. Used to stabilize the blood-retinal barrier and to induce resolution of macular edema.

Triamcinolone (Amcort, Kenalog)

For inflammatory dermatosis responsive to steroids; decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Posterior sub-Tenon injection of steroid to reduce cystoid macular edema. Depending on etiology of edema, it is often DOC.

Dosing

Adult

20 mg via sub-Tenon injection as a single dose; may repeat q4-12wk as indicated

Pediatric

Administer as in adults

Interactions

Coadministration with barbiturates, phenytoin, and rifampin decrease effects of triamcinolone

Contraindications

Documented hypersensitivity; fungal, viral, and bacterial skin infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Multiple complications (eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression) may occur; abrupt discontinuation of glucocorticoids may cause adrenal crisis

Follow-up

Further Outpatient Care

• After scatter laser photocoagulation, monitor the patient for regression or progression of neovascularization. Instruct the patient to contact an ophthalmologist if a decrease or change in vision is noted.

Complications

• Complications of retinal neovascularization can lead to severe visual loss due to persistent vitreous hemorrhage and retinal detachment.
• Anterior segment neovascularization can cause neovascular glaucoma with resultant visual loss and, occasionally, even loss of the eye.
• Peripheral retinal nonperfusion can limit a patient's visual field, and, if the area of nonperfusion extends into the macula, severe visual loss may result. No effective treatment exists to prevent capillary nonperfusion.
• Macular puckering has been reported to occur in 3% of eyes after scatter laser photocoagulation treatment of Eales disease.

Prognosis

• More than 90% of patients can be brought to a morphological standstill with unchanging visual acuity.
• Gieser and Murphy reported that 67% of patients had a final visual acuity in the better eye of 20/40 or better, 24% ranged from 20/50 to 20/200, and 9% were worse than 20/250.¹
• In another study from India, 72% of eyes that underwent vitrectomy for complications of Eales disease maintained a vision of 20/200 or better over 5 years of follow-up care.²

Patient Education

• Patients should be educated to report visual symptoms of floaters or decreased vision to their ophthalmologist as soon as possible, in order to implement effective treatment and to prevent the need of vitrectomy surgery for vitreous hemorrhage or retinal detachment.

Miscellaneous

Medicolegal Pitfalls

• Eales disease is a diagnosis of exclusion and other possible entities causing peripheral retinal neovascularization and vasculitis must be eliminated from the differential diagnosis. An appropriate workup to rule out these conditions is necessary, because many of them are treatable. Such diagnoses include the following:
  • Sickle cell disease
  • Beta thalassemia
  • Small vessel hyalinosis
  • Diabetes mellitus
  • Branch retinal vein or arterial occlusion
  • Retinal embolization
  • Retinopathy of prematurity
  • Familial exudative vitreoretinopathy
  • Hyperviscosity syndromes (eg, 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

Multimedia

Media file 1: Eales disease. Fundus photo of the peripheral retina, revealing vascular tortuosity and peripheral retinal neovascularization.

Media file 2: Eales disease. Fluorescein angiogram of late leakage from peripheral retinal neovascularization in same patient as in Media file 1.

References

1. Gieser AS, Murphy RP. Eales disease. In: Retina. Vol 2. 1994:1503-07.

2. Shanmugam MP, Badrinath SS, Gopal L, Sharma T. Long term visual results of vitrectomy for Eales disease complications. Int Ophthalmol. 1998;22(1):61-4. [Medline].

3. Antigüedad A, Zarranz JJ. [Eales' disease involving central nervous system white matter]. Neurologia. Aug-Sep 1994;9(7):307-10. [Medline].

4. Bhooma V, Sulochana KN, Biswas J, Ramakrishnan S. Eales' disease: accumulation of reactive oxygen intermediates and lipid peroxides and decrease of antioxidants causing inflammation, neovascularization and retinal damage. Curr Eye Res. Feb 1997;16(2):91-5. [Medline].

5. Das T, Namperumalsamy P. Combined photocoagulation and cryotherapy in treatment of Eales' retinopathy. Preliminary report. Indian J Ophthalmol. 1987;35(5-6):108-18. [Medline].

6. Eller AW, Bontempo FA, Faruki H, Hassett AC. Peripheral retinal neovascularization (Eales disease) associated with the factor V Leiden mutation. Am J Ophthalmol. Jul 1998;126(1):146-9. [Medline].

7. Elliot AJ. 30-year observation of patients with Eale's disease. Am J Ophthalmol. Sep 1975;80(3 Pt 1):404-8. [Medline].

8. Gieser SC, Murphy RP. Eales disease. In: Principles and Practice of Ophthalmology. Vol 2. 1994:791-795.

9. Gordon MF, Coyle PK, Golub B. Eales' disease presenting as stroke in the young adult. Ann Neurol. Aug 1988;24(2):264-6. [Medline].

10. Katz B, Wheeler D, Weinreb RN, Swenson MR. Eales' disease with central nervous system infarction. Ann Ophthalmol. Dec 1991;23(12):460-3. [Medline].

11. Kutsal YG, Altioklar K, Atasu S, Kutluk K, Atmaca L. Eales' disease with hemiplegia. Clin Neurol Neurosurg. 1987;89(4):283-6. [Medline].

12. Magargal LE, Walsh AW, Magargal HO, Robb-Doyle E. Treatment of Eales' disease with scatter laser photocoagulation. Ann Ophthalmol. Aug 1989;21(8):300-2. [Medline].

13. Masson C, Denis P, Prier S, Martin N, Masson M, Cambier J. [Eales' disease with neurologic disorders]. Rev Neurol (Paris). 1988;144(12):817-9. [Medline].

14. Pathengay A, Pilli S, Das T. Intravitreal triamcinolone acetonide in Eales' disease: a case report. Eye. Jun 2005;19(6):711-3. [Medline].

15. Phanthumchinda K. Eales' disease with myelopathy. J Med Assoc Thai. Apr 1992;75(4):255-8. [Medline].

16. Renie WA, Murphy RP, Anderson KC, Lippman SM, McKusick VA, Proctor LR, et al. The evaluation of patients with Eales' disease. Retina. Fall-Winter 1983;3(4):243-8. [Medline].

17. Sawhney IM, Chopra JS, Bansal SK, Gupta AK. Eales' disease with myelopathy. Clin Neurol Neurosurg. 1986;88(3):213-5. [Medline].

18. Singhal BS, Dastur DK. Eales' disease with neurological involvement Part 1. Clinical features in 9 patients. J Neurol Sci. Mar 1976;27(3):313-21. [Medline].

19. Spitznas M, Meyer-Schwicherath G, Stephan B. Treatment of Eales' disease with photocoagulation. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1975;194(3):193-8. [Medline].

20. Spitznas M, Meyer-Schwickerath G, Stephan B. The clinical picture of Eales' disease. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1975;194(2):73-85. [Medline].

21. Sulochana KN, Biswas J, Ramakrishnan S. Eales' disease: increased oxidation and peroxidation products of membrane constituents chiefly lipids and decreased antioxidant enzymes and reduced glutathione in vitreous. Curr Eye Res. Sep 1999;19(3):254-9. [Medline].

Keywords

Eales' disease, Eale's disease, idiopathic obliterative vasculopathy, idiopathic recurrent vitreal hemorrhage, periphlebitis retinae

Contributor Information and Disclosures

Author

Daniel B Roth, MD, Assistant Clinical Professor, Department of Ophthalmology, University of Medicine and Dentistry of New Jersey
Daniel B Roth, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
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

CME Editor

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.

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