Abnormal Retinal Vasculature in a Young Child: A Diagnostic Dilemma

Aim: To report the case of a young child who presented with sudden visual loss and characteristic abnormal retinal vasculature.Design: Case ReportMethods: A 13-year-old Indian boy presented with sudden visual loss in the left eye (20/1200) for 1 month, associated with recent-onset of seizures. Ocular examination showed a relative afferent pupillary defect, neovascularization of iris, absence of retinal vasculature beyond second branching, and arterio-venous shunts at the junction of vascular and avascular retina. Fundus fluorescein angiography showed delayed arm-to-retina time, leading edge of fluorescein dye in arteries and peripheral capillary non-perfusion areas.Peripheral pulses were absent and carotid doppler showed bilateral carotid artery occlusion (left one being worse affected), suggestive of Takayasu's arteritis (TA). Despite being started on immunotherapy, he lost complete vision in the left eye.Conclusion: TA may present initially to ophthalmologists, who need to maintain a high index of suspicion for early diagnosis and management of this life-threatening disease.