A new staging system for idiopathic retinal periphlebitis

To develop and standardize a universally acceptable new staging system for idiopathic retinal periphlebitis (Eales disease).

A new staging system was established and standardized based on standard terminology and features. Idiopathic retinal periphlebitis was classified as peripheral and central types. Peripheral disease consisted of four stages. Stage 1 is periphlebitis of small (1a) and large (1b) caliber vessels with superficial retinal hemorrhages. Stage 2a denotes capillary nonperfusion and 2b neovascularization elsewhere/of the disc. Stage 3a is classified as fibrovascular proliferation and 3b vitreous hemorrhage. Stage 4a is traction/combined rhegmatogenous retinal detachment whereas 4b is rubeosis iridis, neovascular glaucoma, complicated cataract, and optic atrophy. A total of 253 cases of idiopathic retinal periphlebitis (mean age, 24.7 +/- 4.7 years, all male) presenting at this tertiary care center were classified prospectively according to the new staging system, by two independent observers (interobserver correlation = 0.7).

The new staging system was consistent, simple, and easy to recall. Peripheral and central types of idiopathic retinal periphlebitis were found in 94.07% and 5.93% of cases, respectively. The new staging system also defined the severity of the disease. Vitreous hemorrhage was found to be the commonest presenting feature (51.68%), whereas traction/combined rhegmatogenous detachment was found in 5.88% of cases.

The new staging system is useful in classifying and assessing the severity of disease. Management strategy can also be defined according to the stage of the disease. It is designed to promote the use of standard assessment with applications to clinical management and research.