Evolution of Peripheral Retinal Perfusion in Sickle Cell Retinopathy During Severe Vaso-Occlusive Crises: A Report of Two Cases

Sickle cell retinopathy (SCR) is characterized by retinal vaso-occlusion and peripheral ischemia.We describe two patients with homozygous sickle cell disease (HbSS) who presented with extensive peripheral retinal nonperfusion during severe vaso-occlusive crises.Multimodal retinal imaging was performed during the acute phase and after systemic stabilization.Following systemic optimization, including exchange transfusion, follow-up fluorescein angiography demonstrated a reduction in peripheral retinal nonperfusion in both patients without progression of neovascularization.These findings suggest that retinal perfusion in SCR may remain dynamic following systemic optimization and raise questions regarding the optimal timing of local retinal treatment.Prospective quantitative studies are needed to further characterize these changes and their potential implications for management.

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Journal
Cureus
Published
2026-10-14
DOI
https://doi.org/10.7759/cureus.117897
Primary Topic
Hemoglobinopathies and Related Disorders
Type
article
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article

Evolution of Peripheral Retinal Perfusion in Sickle Cell Retinopathy During Severe Vaso-Occlusive Crises: A Report of Two Cases

I. Daoudi, Sina Hakami, Florence Andrée Rasquin, Laurence Dominique Postelmans et al.
Cureus
Hemoglobinopathies and Related Disorders
article

Evolution of Peripheral Retinal Perfusion in Sickle Cell Retinopathy During Severe Vaso-Occlusive Crises: A Report of Two Cases

I. Daoudi, Sina Hakami, Florence Andrée Rasquin, Laurence Dominique Postelmans, Iris Païdassi, Elena Missa, Nikola Filipovic
article en

Abstract

Sickle cell retinopathy (SCR) is characterized by retinal vaso-occlusion and peripheral ischemia.We describe two patients with homozygous sickle cell disease (HbSS) who presented with extensive peripheral retinal nonperfusion during severe vaso-occlusive crises.Multimodal retinal imaging was performed during the acute phase and after systemic stabilization.Following systemic optimization, including exchange transfusion, follow-up fluorescein angiography demonstrated a reduction in peripheral retinal nonperfusion in both patients without progression of neovascularization.These findings suggest that retinal perfusion in SCR may remain dynamic following systemic optimization and raise questions regarding the optimal timing of local retinal treatment.Prospective quantitative studies are needed to further characterize these changes and their potential implications for management.

Cureus
Erasmus Hospital (BE), Centre Hospitalier Universitaire Brugmann (BE), Centre Hospitalier Universitaire de Saint-Pierre (BE)
Good health and well-being
Openalex Percentile: Top 14%
Hemoglobinopathies and Related Disorders
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