Title : Beyond the classic IRVAN an atypical presentation of Eales disease
Abstract:
Purpose: To report an unusual case demonstrating overlapping features of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) and Eales' disease, highlighting the diagnostic challenges in atypical retinal vasculitis.
Methods: A 31-year-old male presented with sudden-onset blurring of vision in the left eye for 10 days. Detailed ocular examination, multimodal imaging and systemic evaluation were performed.
Results: Best-corrected visual acuity was 20/20 in the right eye and 20/30 in the left. Fundus revealed optic disc edema, partial macular star, and perivenous sheathing. Capillary aneurysmal dilatations were noted superior to disc. OCT showed peripapillary subretinal fluid, and angiography demonstrated aneurysmal vascular dilatations over the disc, late disc staining, peripheral capillary non-perfusion, and non-leaky capillary dilatations without definite neovascularization. Systemic work-up showed positive Mantoux, raised ESR, a normal IGRA, c-ANCA and normal chest CT. The patient was treated with intravenous methylprednisolone followed by oral steroids, resulting in complete resolution of subretinal fluid, though vascular dilatations persisted. The case fulfilled two major IRVAN criteria (neuroretinitis, vasculitis) and one minor criterion (capillary non-perfusion). Early-phase angiography suggested possible optic nerve head aneurysmal changes, potentially meeting the third major criterion. However, Mantoux positivity and occlusive venous periphlebitis supported an atypical presentation of Eales' disease.
Conclusions: This case represents a rare IRVAN-like variant of Eales' disease—not previously reported. It underscores overlapping phenotypes in retinal vasculitis and emphasizes the need for flexible diagnostic interpretation, multimodal imaging, and long-term follow-up to guide individualized management.