Eales Disease - Rare Eye Disorder, Misdiagnosed Eye Diseases

Eales Disease

Eales Disease is a rare disorder of sight that appears as an inflammation and white haze around the outercoat of the veins in the retina. The disorder is most prevalent among young males and normally affects both eyes. Usually, vision is suddenly blurred because the clear jelly that fills the eyeball behind the lens of the eye seeps out (vitreous hemorrhaging).

The cause of Eales disease is unknown. It is a diagnosis of exclusion and is thought to be idiopathic. No causative drugs, environmental factors, or infectious agents have been identified. Although a hypersensitivity to tuberculin protein has been reported, no clear relationship to tuberculosis has been found.

Peak age of onset is 20-35 years, with a reported range of 13-63 years.

History

Eales' disease was first described by the British ophthalmologist Henry Eales in 1880 (Eales 1880). Eales' disease is an idiopathic, obliterative vasculopathy, which typically affects the peripheral retina of otherwise healthy young adults. Retinal changes include vasculitis, extensive peripheral non-perfusion, and neovascularization. Characteristically, visual loss is caused by bilateral recurrent vitreous haemorrhages (Gieser et al. 2001).

The only countries with reported high incidences of Eales' disease are India and certain countries in the Middle East. Eales' disease is rare in North America (Magargal et al. 1989; Gieser et al. 2001; Biswas et al. 2002). The incidence of the disease in Denmark is also very low, with approximately one case per year seen in our clinic, which draws from an urban area of 1.2 million people. As far as we know, no previous cases of Eales' disease have been reported in Greenland. We report four cases of presumed Eales' disease diagnosed within a 6.5-year period in Inuit, all of whom were born in Greenland.

Symptoms

Most patients present with symptoms of floaters, specks, cobwebs, blurring, or decreased vision associated with vitreous hemorrhage. Other patients have blurring associated with retinal vasculitis or uveitis, but without hemorrhage. Often, patients complain of uniocular symptoms, but ophthalmic examination reveals early changes of Eales disease in the other eye. Bilateral involvement is evident in 80-90% of patients.

Treatment

Intravitreal steroids may facilitate treatment of Eales' disease and with scatter laser photocoagulation. Treatments include thyroid extract, osteogenic hormones, androgenic hormones, and systemic steroids. The antioxidant vitamins A, C, and E have been suggested recently as a possible therapy because antioxidizing enzymes are deficient in the vitreous samples of patients with Eales disease.