CASE REPORT
A 39-years-old man presented to a clinic with complaints of decreased blurred vision and floaters in his left eye over the previous two weeks.
His best-corrected visual acuity at the initial presentation was 20/ 20 in the right eye and hand movement in the left eye. The anterior segment and intraocular pressure were normal.
The visual field using the Goldmann perimeter and the Humphrey static achromatic automatic perimetry (central 30-2 threshold test) was normally at the right eye and could not be performed on the left eye owing to the markedly decreased visual acuity.
Ultrasonography of the left eye highlighted dense vitreous floaters, which obscured the view of the fundus. Dilated ocular fundus examination of the right eye showed pallor of the optic nerve head; temporally from the macula in the mid-periphery, there were perivascular sheathing, scattered wet perivascular exudates, dotted and flame-shaped intraretinal hemorrhages, focal occlusions of retinal vessels, extensive nonperfused areas of the capillaries from the macula in the mid-retina periphery, which extended towards the posterior pole and retinal periphery, giving rise to preretinal neovascularization prominent into the vitreous in the fashion of a sea fan.
Taking into account all the clinical, angiographic, and ultrasonographic assessments, the diagnoses of bilateral Eales disease in the late (proliferative) stage with neovascularization and retinal and vitreous hemorrhages were established.
DISEASE
Eales’ disease was first described by British ophthalmologist Henry Eales in 1880. Eales’ disease is an idiopathic occlusive vasculitis involving the mid-peripheral retina that is characterized by retinal venous inflammation (periphlebitis), vascular occlusion, and subsequent retinal neovascularization.
A hallmark of Eales’ disease is recurrent vitreous hemorrhage.
Eales’ disease mainly affects young males, with a mean age at presentation of around 30 years. It is more prevalent in India and Middle Eastern countries with cases observed worldwide.
In a study by Biswas et al., a male-to-female ratio of 20:1 was observed with a mean age at presentation of 29.9 years, with a range from 11– 59 years.
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From Choroida — the team behind this siteMANAGEMENT
General Treatment
The management of Eales’ disease depends upon the stage of the disease. The treatment strategy consists of medical treatment with oral corticosteroids in the active inflammatory stage and laser photocoagulation for retinal ischemia and neovascularization.
Biswas et al. observed that the timely use of oral corticosteroid during active inflammation and the use of laser ablation of areas with capillary non-perfusion had a statistically significant beneficial impact on visual outcomes during a 10-year follow-up period.
Medical Treatment
- Corticosteroids
- Anti-Vascular Endothelial Growth Factor (Anti-VEGF)
- Anti-Tuberculosis Treatment
- Photocoagulation
- Surgical treatment
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From Choroida — the team behind this siteREFERENCES
- Eales H. Primary retinal hemorrhage in young men. Ophthalmic Rev. 1882;1: 41.
- Biswas J, Ravi RK, Naryanasamy A, Kulandai LT, Madhavan HN. Eales’ disease – current concepts in diagnosis and management. J Ophthalmic Inflamm Infect. 2013;3(1):11.
- Das T, Pathengay A, Hussain N, Biswas J. Eales’ disease: diagnosis and management. Eye (Lond). 2010;24(3):472-82.
- Biswas J, K R R, Pal B, Gondhale HP, Kharel Sitaula R. Long-Term Outcomes of a Large Cohort of Patients with Eales’ Disease. Ocul Immunol Inflamm. 2018;26(6):870‐876.
- Ishaq M, Karamat S, Niazi MK. HLA typing in patients of Eales disease. J Coll Physicians Surg Pak. 2005 May; 15(5):288-90.
- Rajesh M, Sulochana KN, Coral K, et al. Determination of carbonyl group content in plasma proteins as a useful marker to assess impairment in antioxidant defense in patients with Eales’ disease. Indian J Ophthalmol. 2004;52(2):139-144.
Test yourself
A few questions straight from this article.
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How is Eales' disease best defined?
Eales' disease is an idiopathic occlusive vasculitis of the mid-peripheral retina, marked by retinal venous inflammation, vascular occlusion and later neovascularization. -
What is regarded as the hallmark of Eales' disease?
Recurrent vitreous hemorrhage is described as the hallmark of Eales' disease, following the periphlebitis and vascular occlusion that define it. -
What male-to-female ratio was observed in the Biswas study of Eales' disease?
Biswas and colleagues observed a male-to-female ratio of 20 to 1 in their cohort of patients with Eales' disease. -
In which regions is Eales' disease reported to be more prevalent?
Although cases are seen worldwide, Eales' disease is more prevalent in India and in Middle Eastern countries. -
Which fundus appearance describes the preretinal neovascularization of proliferative Eales' disease?
In the proliferative stage the preretinal new vessels project prominently into the vitreous in the fashion of a sea fan, arising from extensive non-perfused retina. -
What primarily guides the choice of treatment in Eales' disease?
Management of Eales' disease depends upon the stage, with anti-inflammatory treatment for active disease and laser for ischemia and neovascularization. -
Which treatment is used during the active inflammatory stage of Eales' disease?
Medical treatment in the active inflammatory stage rests on oral corticosteroids, with laser reserved for retinal ischemia and neovascularization. -
How did the reported patient with Eales' disease first present?
The 39-year-old man presented with decreased blurred vision and floaters in the left eye over the preceding two weeks, with hand movement acuity on that side. -
Which systemic therapy is listed among the medical treatments for Eales' disease?
The medical treatment options listed for Eales' disease include corticosteroids, anti-VEGF agents and anti-tuberculosis treatment, alongside photocoagulation and surgery. -
Over a 10-year follow-up, which approach significantly improved visual outcomes in Eales' disease?
Biswas and colleagues found that timely oral corticosteroid during active inflammation, combined with laser ablation of areas of capillary non-perfusion, had a statistically significant benefit on vision.
