Acute macular infarctions in pediatric patients with hemoglobin SS disease

Patrick Sy Lee; Cesar Alfaro; Rao Me; Xihui Lin

doi:10.1016/j.ajoc.2023.101913

Acute macular infarctions in pediatric patients with hemoglobin SS disease

Am J Ophthalmol Case Rep. 2023 Aug 17:32:101913. doi: 10.1016/j.ajoc.2023.101913. eCollection 2023 Dec.

Authors

Patrick Sy Lee¹, Cesar Alfaro¹, Rao Me¹, Xihui Lin¹

Affiliation

¹ Kresge Eye Institute/Department of Ophthalmology, Visual and Anatomical Sciences, Wayne State University School of Medicine, 4717 St. Antoine Street, Detroit, MI, 48201, USA.

Abstract

Purpose: To report two cases of symptomatic posterior pole arterial occlusions in patients with hemoglobin SS disease.

Observations: Two teenage patients with hemoglobin SS disease presented with visual distortions, and on dilated fundus examination and testing, they were found to have arterial occlusions involving the posterior pole. The patients were evaluated for stroke with head imaging and received exchange transfusion by hematology.

Conclusions and importance: This case series reports the unusual findings of arterial occlusions in the posterior pole resulting in areas of retinal whitening and ischemia in patients with HbSS. While sickle cell retinopathy is typically considered a peripheral retinal disease, these findings underscore the importance of vigilance when examining patients with sickle cell disease.

Keywords: Hemoglobin S; Ischemia; Macula; Sickle cell disease; Sickle cell retinopathy.

Publication types

Case Reports