Eales disease


Primary idiopathic peripheral obstructive retinal vasculopathy of unclear etiology characterized by three overlapping stages of venous inflammation, ischemia, and retinal neovascularization, being hallmarked by recurrent retinal and vitreal hemorrhages.

History:

Eales disease was considered a form of vasomotor neurosis by Sir Henry Eales who, in 1880, described five young men with recurring vitreous and retinal hemorrhages associated with constipation and epistaxis, but it was Wardsworth in 1887 who explained the disease’s association with retinal inflammation


Etiopathogenesis

Type III hypersensitivity to tuberculin proteins:

  • Tuberculosis exposure
  • Hypersensitivity to tuberculoprotein and tubercle bacilli

Disease course:

Characterised by presence of 3 overlapping stages.
  • Early inflammatory stage:
    • Periphlebitis (HALLMARK)
    • Venous dilatation
    • Perivascular exudates
  • Middle ischemic stage:
    • Capillary ischemia
    • Demarcation between perfused and non-perfused zones is marked by veno-venous shunts, venous beadings, and microaneurysms
  • Late proliferative stage:
    • Neovascularization (at junction between perfused and non-perfused retina) that leads to recurrent vitreous hemorrhages with/without retinal detachment
Fundus photograph of Eales' disease with active periphlebitis
Fundus photograph of Eales’ disease with active periphlebitis | Biswas, J., Ravi, R. K., Naryanasamy, A., Kulandai, L. T., & Madhavan, H. N. (2013). Eales’ disease – current concepts in diagnosis and management. Journal of ophthalmic inflammation and infection, 3(1), 11. https://doi.org/10.1186/1869-5760-3-11

Saxena and Kumar staging system:

  • I: Periphlebitis of small (Ia) and large (Ib) caliber vessels with superficial retinal hemorrhages
  • IIa: Capillary nonperfusion
  • IIb: Revascularization elsewhere/of the disc
  • IIIa: Fibrovascular proliferation
  • IIIb: Vitreous hemorrhage
  • IVa: Traction/combined rhegmatogenous retinal detachment
  • IVb: Rubeosis iridis, neovascular glaucoma, complicated cataract, and optic atrophy

Clinical features

  • Sudden painless loss of vision
  • Floaters

Diagnosis

Eales disease is a diagnosis of exclusion. In the absence of other systemic conditions, peripheral retinal inflammation and recurrent vitreous hemorrhages are important defining features.

Fluorescein angiography:

  • Leakage of the sheathed vessels, retinal vascular tortuosity and telangiectasias, shunt vessels, venous stasis, capillary non-perfusion, retinal neovascularization

Management

Early management:

  • Steroidal and non-steroidal agents
  • Intravitreal anti-vascular endothelial growth factor (VEGF) therapy
  • Anti-tubercular therapy (cases with exposure to TB)

Late-stage management:

  • Laser scatter retinal photocoagulation (peripheral pan retinal photocoagulation)

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