Eales Disease 

  • Author: Daniel B Roth, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 25, 2010
 

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 retiEales 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.

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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.[1]

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, University of Medicine and Dentistry of New Jersey Robert Wood Johnson Medical School, Retina Vitreous Center

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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.

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians & Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

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; OptiMedica Ownership interest Consulting

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.

References

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

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

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

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

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

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

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

  8. 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].

  9. 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].

  10. .

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

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

  13. Moyenin P, Grange JD. [Eales' syndrome. Clinical aspects, therapeutic indications and course of 29 cases]. J Fr Ophtalmol. 1987;10(2):123-8. [Medline].

  14. Küçükerdönmez C, Akova YA, Yilmaz G. Intravitreal injection of bevacizumab in Eales disease. Ocul Immunol Inflamm. Jan-Feb 2008;16(1):63-5. [Medline].

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

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

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

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

  23. 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].

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

  25. 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].

  26. 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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