Eales’ Disease

Overview

Eales' disease is characterised by retinal vasculitis and neovascularisation resulting in recurrent vitreous haemorrhage. It affects the peripheral retina and is most common in young Indian males.

The cause of Eales' disease is unclear and it is typically thought to be idiopathic. Inflammation of the peripheral blood vessels (vasculitis) causes obstruction and sheathing of these vessels. This leads to ischaemia within the are of retinal supplied by the obstructed vessels, stimulating the release of vascular endothelial growth factor (VEGF) which in turn stimulates neovascularisation. Tractional retinal detachment, vitreous and retinal haemorrhages follow.

In the active stage, perivascular exudates (cuffing) of the vessels occurs, primarily affecting the veins in the retinal periphery. Exudation accompanies the vascular inflammation.

In the inactive stage, sheathing of veins, sclerosis and pigmentation of venules, and irregular vessel caliber may be noted.

Case Examples

  • Case 1

    A 70-year-old Caucasian male with type 2 diabetes, diagnosed 11 years previously. His latest HbA1c was 8% however it has been as high as 12% in the past. He has previously had a retinal vein occlusion and a vitreous haemorrhage. He was previously diagnosed with Eales' disease in the right eye. His best corrected visual acuity in this eye was 6/15 (20/50).

    This case will focus on the right eye only.

    Fundus photograph and red-free image (right eye)

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    Fundus photograph and red-free image (right eye)

    Retinal photos show extensive peripapillary atrophy, an area of chorioretinal atrophy inferonasal to the optic disc and blot haemorrhages within the superior vascular arcade.

    Optomap widefield and green separation images (right eye)

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    Optomap widefield and green separation images (right eye)

    Ghost vessels are visible throughout the mid-peripheral and peripheral fundus, most notable in the supero-nasal retina. There are numerous areas of chorioretinal atrophy and RPE hyperplasia changes, scattered blot haemorrhages, hard exudates in the superotemporal macula, and areas of opacified vitreous strands temporally.

    Fundus autofluorescence imaging (right eye)

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    Fundus autofluorescence imaging (right eye)

    The areas of choroiretinal atrophy are hypo-autofluorescent, as are the areas of RPE hyperplasia and retinal haemorrhages.

    Spectralis OCT volume and line scans (right macula)

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    Spectralis OCT volume and line scans (right macula)

    OCT showed evidence of cystoid macular oedema just superior to the fovea. Further superiorly, hard exudates can be seen as numerous hyper-reflective intraretinal deposits. Abnormal vitreoretinal adhesions are visible in the inferior frames of the volume scan.

    OCT Angiography (right eye, 6x6mm superficial vascular plexus)

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    OCT Angiography (right eye, 6x6mm superficial vascular plexus)

    OCTA shows mild capillary dropout and reduced retinal perfusion inferior to the macula. Superior to the foveal vascular zone (FAZ) are some anomalous vessels within superficial vascular layers of the inner retina.

Differential Diagnosis

Sickle cell disease

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Coats' disease

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Familial exudative vitreoretinopathy

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Proliferative diabetic retinopathy

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Chronic branch retinal vein occlusion

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References

Biswas, J., Ravi, R.K., Naryanasamy, A. et al. (2013) Eales' disease - current concepts in diagnosis and management. J Ophthal Inflamm Infect 3, 11.

Biswas,J. Sharma,T. Gopal,L. Madhavan, HN, Sulochana, KN, Ramakrishnan,S. (2002) Eales Disease—An Update, Survey of Ophthalmology, Volume 47, Issue 3 pages 197-214.