Ischemic retinopathy and uveitis in a patient with tetralogy of Fallot.

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.