Eales Disease Clinical Presentation
- Author: Daniel B Roth, MD; Chief Editor: Hampton Roy, Sr, MD more...
Most Eales disease patients present with symptoms of floaters, specks, cobwebs, blurring, or decreased vision associated with vitreous hemorrhage. Other Eales disease patients have blurring associated with retinal vasculitis or uveitis, but without hemorrhage. Often, patients report uniocular symptoms, but ophthalmic examination reveals early changes of Eales disease in the other eye. Bilateral involvement is evident in 80-90% of patients.
The physical findings in Eales disease 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,[3, 4] ischemic stroke, hemiplegia, and multifocal white matter abnormalities have been reported.[7, 8] 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.
The cause of Eales disease is unknown. Eales disease is a diagnosis of exclusion and is thought to be idiopathic. No causative drugs, environmental factors, or infectious agents for Eales disease have been identified. Although a hypersensitivity to tuberculin protein has been reported, no clear relationship to tuberculosis has been found. An 88-kd acute phase reactant protein has been found in patients with Eales disease that is immunologically identical to that found in patients with posterior uveitis, tuberculosis, leprosy, and rheumatoid arthritis. The role of this protein is yet undetermined.
Increasing data implicate interleukin (IL)–1 and tumor necrosis factor-alpha (TNFα) in the inflammatory stage of Eales disease. Genetic polymorphism studies suggest low IL-10 expression and high TNFα may increase the occurrence and severity of Eales.
Singh R, Toor P, Parchand S, Sharma K, Gupta V, Gupta A. Quantitative polymerase chain reaction for Mycobacterium tuberculosis in so-called Eales' disease. Ocul Immunol Inflamm. 2012 Jun. 20(3):153-7. [Medline].
Gieser AS, Murphy RP. Eales disease. Retina. 1994. Vol 2: 1503-07.
Phanthumchinda K. Eales' disease with myelopathy. J Med Assoc Thai. 1992 Apr. 75(4):255-8. [Medline].
Sawhney IM, Chopra JS, Bansal SK, Gupta AK. Eales' disease with myelopathy. Clin Neurol Neurosurg. 1986. 88(3):213-5. [Medline].
Gordon MF, Coyle PK, Golub B. Eales' disease presenting as stroke in the young adult. Ann Neurol. 1988 Aug. 24(2):264-6. [Medline].
Kutsal YG, Altioklar K, Atasü S, Kutluk K, Atmaca L. Eales' disease with hemiplegia. Clin Neurol Neurosurg. 1987. 89(4):283-6. [Medline].
Antiguedad A, Zarranz JJ. [Eales' disease involving central nervous system white matter]. Neurologia. 1994 Aug-Sep. 9(7):307-10. [Medline].
Katz B, Wheeler D, Weinreb RN, Swenson MR. Eales' disease with central nervous system infarction. Ann Ophthalmol. 1991 Dec. 23(12):460-3. [Medline].
Saxena S, Pant AB, Khanna VK, Agarwal AK, Singh K, Kumar D. Interleukin-1 and tumor necrosis factor-alpha: novel targets for immunotherapy in Eales disease. Ocul Immunol Inflamm. 2009 May-Jun. 17(3):201-6. [Medline].
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].
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].
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].
Agrawal S, Agrawal J, Agrawal TP. Intravitreal triamcinolone acetonide in Eales disease. Retina. 2006 Feb. 26(2):227-9. [Medline].
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].
Moyenin P, Grange JD. [Eales' syndrome. Clinical aspects, therapeutic indications and course of 29 cases]. J Fr Ophtalmol. 1987. 10(2):123-8. [Medline].
Kucukerdonmez C, Akova YA, Yilmaz G. Intravitreal injection of bevacizumab in Eales disease. Ocul Immunol Inflamm. 2008 Jan-Feb. 16(1):63-5. [Medline].
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].
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].
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].
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].
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].
Elliot AJ. 30-year observation of patients with Eale's disease. Am J Ophthalmol. 1975 Sep. 80(3 Pt 1):404-8. [Medline].
Gieser SC, Murphy RP. Eales disease. Principles and Practice of Ophthalmology. 1994. 2: 791-795.
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].
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].
Renie WA, Murphy RP, Anderson KC, et al. The evaluation of patients with Eales' disease. Retina. 1983 Fall-Winter. 3(4):243-8. [Medline].
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].
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].
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].