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Eales Disease

  • Author: Daniel B Roth, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Aug 21, 2014
 

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 in Eales disease 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. Note the clinical image below:

Eales disease. Fundus photo of the peripheral reti Eales disease. Fundus photo of the peripheral retina, revealing vascular tortuosity and peripheral retinal neovascularization.
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Pathophysiology

The pathophysiology of Eales disease is mostly unknown. Eales disease 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.[1]

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Epidemiology

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 of Eales disease; however, a study of 55 patients by Gieser and Murphy found that men and women are affected equally.[2]

Age

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

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Contributor Information and Disclosures
Author

Daniel B Roth, MD Assistant Professor, Department of Ophthalmology, Retina Vitreous Center, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School

Daniel B Roth, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists, Retina Society, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Howard F Fine, MD, MHSc Partner, Associated Retina Consultants, Retina Vitreous Center, PA; Co-founder and Chairman of Scientific Advisory Board, Auris Surgical Robotics, Inc

Howard F Fine, MD, MHSc is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department 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, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

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, Macula Society, Retina Society, Club Jules Gonin

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

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, American Society of Retina Specialists

Disclosure: Nothing to disclose.

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

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  10. Sen A, Paine SK, Chowdhury IH, Mondal LK, Mukherjee A, Biswas A. Association of interferon-gamma, interleukin-10, and tumor necrosis factor-alpha gene polymorphisms with occurrence and severity of Eales' disease. Invest Ophthalmol Vis Sci. 2011 Jan. 52(1):171-8. [Medline].

  11. 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. 1997 Feb. 16(2):91-5. [Medline].

  12. 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. 1999 Sep. 19(3):254-9. [Medline].

  13. Agrawal S, Agrawal J, Agrawal TP. Intravitreal triamcinolone acetonide in Eales disease. Retina. 2006 Feb. 26(2):227-9. [Medline].

  14. Chanana B, Azad RV, Patwardhan S. Role of intravitreal bevacizumab in the management of Eales' disease. Int Ophthalmol. 2010 Feb. 30(1):57-61. [Medline].

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  17. Kumar A, Sinha S. Rapid regression of disc and retinal neovascularization in a case of Eales disease after intravitreal bevacizumab. Can J Ophthalmol. 2007 Apr. 42(2):335-6. [Medline].

  18. Patwardhan SD, Azad R, Shah BM, Sharma Y. Role of intravitreal bevacizumab in Eales disease with dense vitreous hemorrhage: a prospective randomized control study. Retina. 2011 May. 31(5):866-70. [Medline].

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  20. 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].

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

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

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

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

  25. 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].

  26. Renie WA, Murphy RP, Anderson KC, et al. The evaluation of patients with Eales' disease. Retina. 1983 Fall-Winter. 3(4):243-8. [Medline].

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

  28. 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].

  29. 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].

 
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Eales disease. Fundus photo of the peripheral retina, revealing vascular tortuosity and peripheral retinal neovascularization.
Eales disease. Fluorescein angiogram of late leakage from peripheral retinal neovascularization.
 
 
 
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