Abstract
PURPOSE: We present a case of unilateral exudative retinal detachment and optic disc edema with choroidal hemangioma in a patient concurrently diagnosed with Transposition of the Great Arteries (TGA) and Sturge-Weber Syndrome (SWS). OBSERVATIONS: A seven-year-old patient presented with complaints of headache, photophobia, periocular pain, and diminished vision, accompanied by redness and lid swelling following a febrile illness. Ophthalmic examination revealed abnormally branched episcleral vasculature, exudative retinal detachment, optic disc edema and radiologically evident thickening of the choroid of the right eye. Systemic evaluation revealed hypoxia with cyanosis and clubbed fingers. The patient underwent cardiac interventions, including Fontan and Bidirectional Glenn procedures, aimed at improving systemic oxygenation. The patient's condition showed remarkable improvement following cardiac surgery. The surgical interventions not only improved systemic oxygenation but also resulted in the amelioration of ocular symptoms and visual improvement. Ophthalmic improvements included the resolution of abnormally branched episcleral vasculature, regression of exudative retinal detachment, and reduction in optic disc edema. CONCLUSION AND IMPORTANCE: This case highlights the intricate relationship between hemodynamic and vascular abnormalities of the eye and the clinical manifestations observed in patients presenting with the unique combination of SWS and TGA.