MACULAR INFARCTION IN A PATIENT WITH SICKLE CELL TRAIT

Sickle cell trait affects 8% of African Americans. Once believed to represent a benign carrier state, it has been linked to an increased risk of several of the pathological conditions that arise in sickle cell disease in at-risk individuals with hematologic and vascular comorbidities. Macular infarction is a known complication of sickle cell disease; this article illustrates this unique presentation in a patient with sickle cell trait.

Case report.

A 74-year-old African American man presented with the complaint of a central scotoma of the right eye.

Multimodal retinal imaging identified central macular infarction with severe inner retinal atrophy. Laboratory testing confirmed the presence of sickle cell trait. Other pertinent positives included low levels of protein C and protein S, untreated obstructive sleep apnea, and elevated levels of homocysteine in the setting of alcoholic liver cirrhosis and chronic kidney disease.

Ocular manifestations of sickle cell trait have most frequently been reported in individuals with systemic medical comorbidities that predispose to erythrocyte sickling and vaso-occlusive disease. This case identifies a novel complication of sickle cell trait disorder, macular infarction, in a patient with comorbid associations.