Sickle cell crisis presenting as a masquerade syndrome complicated by macular ischemia

To report the case of a young boy, homozygous for the hemoglobin S, who presented a pseudouveitis in the setting of severe sickle cell retinopathy complicated by macular infarction.

Case report.

A 15 year-old boy with a history of hypertensive uveitis of two months duration was reffered to our institution. He was treated with topical prednisolone acetate, beta-blockers and acetazolamide.The visual acuity was 20/200 RE and 20/25 LE. Anterior inflammation included fine inferior keratic precipitates with 2+ cells RE and 1+ cells LE. Vitreous haze was 2+ preventing clear view of subretinal infiltrates scattered around the posterior pole and midperipheral retina, some of them having a salmon patch appearance. Fluorescein angiograms revealed multiple preretinal haemorrahge and some areas of retinal ischemia. Fundus examination of the left eye was normal. A diagnosis of panuveitis was done and a sickle cell retinopathy was suspected. Systemic workup showed an hemoglobin at 8,2 mg/dl and sickle cells on direct examination. Two days later he developed sudden loss of vision in the right eye. Funduscopy an angiogram revealed macular infarction with occlusion of the retinal arterioles surrounding the foveal avascular zone. The clinical picture was not improved by erythrocyte transfusion. Intraocular pressure raised again after few days, and was finally controlled by anterior chamber paracentesis. The patient was later found to be homozygous for HbS.

Sickle cell retinopathy can rarely masquerade as panuveitis, and can lead to severe ocular complications as an irreversible macular ischemia.