Acute macular infarctions in pediatric patients with hemoglobin SS disease

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.

Fig. 1

Fundus photography in patient 1 showing multiple foci of whitening involving macula bilaterally.

Fig. 2

Fluorescein angiography in patient 1 showing multiple areas of peripheral arterial occlusion with vascular dropout bilaterally.

Fig. 3

OCT of macula in patient 1 showing significant temporal thinning of inner retinal layers, particularly pronounced in left eye.

Fig. 4

Fundus photography in patient 2 showing area of whitening inferotemporally to macula in left eye.

Fig. 5

Fluorescein angiography in patient 2 showing multiple areas of peripheral arterial occlusion with vascular dropout bilaterally. Note focal area of nonperfusion corresponding to area of whitening in Fig. 4.

Fig. 6

OCT of macula in patient 2 showing whitening of inner retinal layers temporal to macula in left eye.