Systemic thrombolysis for acute central retinal artery occlusion in sickle cell disease: Case report

In sickle cell disease (SCD), blood oxygen content is decreased due to anemia and the Hb-SS phenotype, in particular leads to an increased blood viscosity, which limits tissue oxygen delivery. Nonetheless, vasculopathy, correlating with daytime oxygen saturation,¹ thrombophilia and hyper coagulability are all underappreciated etiologies of stroke in SCD.² As a result, there is less known about the role of systemic thrombolysis for the management of acute stroke in SCD. Given the lack of studies and cases reviewed in literature, we describe a patient with SCD found to have an acute stroke treated with both intravenous (IV) alteplase and exchange transfusion.

We describe a 42-year-old African- American man with SCD (HbSS) who presented with an acute central retinal artery occlusion (CRAO) within an hour of onset and subsequently received IV thrombolysis with alteplase. His labs were significant for HbS 91%. He had no central vascular access to undergo emergent red blood cell (RBC) exchange so interventional radiology was consulted to minimize his bleeding risk after receiving tissue plasminogen activator (tPA). A right internal jugular catheter was placed with fluoro-guidance, resulting in minimal blood loss. After two sessions of RBC exchange, his HbS decreased to 26%. He reported an improvement of vision in his left eye the day following acute management and followed up with Hematology for secondary stroke prevention.

In reviewing this case, we recommend that current clinical trials for management of acute stroke including an acute CRAO carefully consider including patients with sickle cell disease to receive simultaneous IV thrombolysis and RBC exchange. The benefits outweigh the risk of a permanent disabling deficit with significant functional impairment. And while considering care plans tailored to the projected needs of acute care management for this patient population, we also recommend implementing healthcare models that improve access to preventative care in settings where the majority of children and adults with SCD live.