Purpose: To describe ischemic retinopathy in a patient with tetralogy of Fallot.
Design: Interventional case report.
Testing: Clinical and imaging evaluation.
Main outcome measures: Clinical, imaging, and laboratory findings in a patient with tetralogy of Fallot.
Results: A 20-year-old female patient with tetralogy of Fallot had progressive visual loss of 3 weeks duration. Bilateral examination revealed dilated, tortuous, conjunctival vessels; prominent anterior chamber reaction; iris neovascularization; posterior synechia; retinal vascular tortuosity in both eyes; and inferior exudative retinal detachment. Fluorescein angiography revealed delayed retinal and choroidal filling. The working diagnosis was ischemic retinopathy with uveitis. The patient was treated for 6 months with a high-dose oral corticosteroid combined with a topical corticosteroid, a topical mydriatic, and panretinal photocoagulation. Conjunctival, vascular congestion subsided with a decrease in anterior chamber reaction. The inferior, exudative retinal detachment resolved, and vision was restored.
Conclusions: Retinal ischemic syndrome combined with uveitis can develop in patients with tetralogy of Fallot. Treatment can restore vision in such patients.