Primary branch retinal artery occlusion in idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome associated with hyperhomocysteinemia.

Purpose: The purpose of this study was to report primary branch retinal artery occlusion in a case with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

Methods: Review of medical case records, color fundus photographs, and fundus fluorescein angiography of a 23-year-old man diagnosed with idiopathic retinal vasculitis, aneurysms, and neuroretinitis.

Patient: A 23-year-old man presented with sudden painless decreased vision in right eye since 1 day. Ocular examination revealed a best-corrected visual acuity of counting fingers 1 feet in the right eye and 20/20 in the left eye, relative afferent pupillary defect in the right eye, 1+ vitreous cells in both eyes, optic disk neovascularization with massive peripapillary and perivascular lipid exudation, and occluded smaller vessels in both eyes. In addition, there was an area of retinal opacification in the posterior pole along the inferotemporal arcade in the right eye. The patient underwent color fundus photography, fluorescein angiography, and a detailed systemic workup.

Results: Fundus fluorescein angiographic features were suggestive of idiopathic retinal vasculitis, aneurysms, and neuroretinitis in both eyes with branch retinal artery occlusion in the right eye. Detailed systemic workup revealed raised serum homocysteine levels. The patient underwent scatter retinal photocoagulation in both eyes and also was started on folic acid and pyridoxine supplementation. At 1 year of follow-up, the best-corrected visual acuity in the right eye improved to 20/60. Posterior segment examination showed reduced exudation, resolution of optic disk neovascularization in both eyes, and clearing of retinal opacification in the right eye. Also, the levels of serum homocysteine decreased over 1 year.

Conclusion: Primary branch retinal artery occlusion can be an atypical presentation of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. Such a case should be thoroughly investigated for underlying hypercoagulable state. Also, a careful long-term follow-up is required for these patients to prevent any neovascularization sequelae.