Jyotirmay Biswas, MD
DIFFERENTIAL DIAGNOSIS AND INVESTIGATION
Eales’ disease was first described by Henry Eales in 1880 (1). The patient presents
with retinal perivasculitis predominantly affecting the peripheral retina (inflammatory
stage), then sclerosis of retinal veins indicating retinal ischemia (ischemic stage), and
finally retinal or optic disk neovascularization, recurrent vitreous hemorrhage with or
without retinal detachment (proliferative stage) (2–4).
The disease is seen more commonly in the Indian subcontinent and the Middle
Eastern countries. It commonly affects healthy young males. The predominant age of
onset of symptoms is between 20 and 30 yr (5).
Patients are often asymptomatic in the initial stages of retinal perivasculitis. Some
patients may develop symptoms such as floaters, blurring of vision, or even gross diminu-
tion of vision due to massive vitreous hemorrhage. Vision in these patients can be normal
to hand movements or light perception only. Bilaterality is quite common (50–90% of
patients) (2,3). Clinical manifestation of this disease is due to three basic pathological
From: Ophthalmology: Ocular Angiogenesis: Diseases, Mechanisms, and Therapeutics
Edited by: J. Tombran-Tink and C. J. Barnstable © Humana Press Inc., Totowa, NJ
changes: inflammation (peripheral retinal perivasculitis); ischemic changes (peripheral
retinal capillary nonperfusion); and neovascularization of the retina or disk, which often
leads to vitreous hemorrhage as well as multiple superficial retinal hemorrhages.
Anterior uveitis is uncommon in Eales’ disease. However, in the severe active
periphlebitis stage, spillover anterior uveitis may occur. Such anterior uveitis is always
nongranulomatous. The presence of granulomatous anterior uveitis should lead one to
suspect sarcoid uveitis, which mimics Eales’ disease. Hypopyon is not seen in Eales’
disease, and hypopyon with retinal vasculitis may indicate Behçet’s disease (6) 6 .
Ophthalmoscopic findings in Eales’ disease often vary and depend on the stage of
the disease. Arterioles are sometimes affected along with the veins. Typically, active
perivasculitis with exudates around the retinal veins is seen involving one or more
quadrants. Such exudates are often found to be associated with superficial retinal
hemorrhages (Fig. 1).
Healed perivasculitis is often seen as the sheathing of the retinal veins. Other vascu-
lar changes includes sclerosed cord of venules, irregularity of vein caliber, pigmenta-
tion along venules, kinky venules, abnormal vascular anastomosis, and veins pulled
into the vitreous cavity (2,3,7) 7 .
Active or healed choroiditis is not seen in Eales’ disease. However, a few small
chorioretinal atrophic patches close to the retinal vessels are seen (7) 7 .
Central retinal periphlebitis is markedly uncommon compared with peripheral retinal
periphlebitis (2,3,8). Such central involvement is often limited to one or more venous
trunks. This is classified as central Eales, a variant of classical Eales’disease (8). Macular
changes are relatively uncommon (9). The most common macular change seen is macular
edema. Other changes included exudates in the macula and epimacular membrane.
Peripheral retinal neovascularization of the retina is quite frequently seen in Eales’
disease (2,3,7) 7 (Fig. 2). Optic disk neovascularization is significantly uncommon (2–4).
Dense vitritis is uncommon in Eales’ disease. However, mild overlying vitreous haze
Fig. 1. Montage fundus photograph of a case of Eales’ disease showing multiple patches
of active retinal periphlebitis. See color version on companion CD.
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