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Updated: Feb 26, 2026
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New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review
Daniel A Pahl1, Nancy S Green, Monica Bhatia
1*Departments of Pediatrics †Ophthalmology, Columbia University Medical Center, New York, NY.
Insights
New imaging techniques reveal higher prevalence of early sickle retinopathy in children with sickle cell disease. Early detection through advanced methods like OCT angiography and ultra-widefield fluorescein angiography aids understanding and treatment.
Area of Science:
- Ophthalmology
- Pediatrics
- Hematology
Background:
- Sickle retinopathy is eye vascular damage from sickle cell disease, potentially causing vision loss.
- It affects children with sickle cell disease, with traditional screening showing ~10% prevalence.
Purpose of the Study:
- To review sickle retinopathy in children, covering epidemiology, risk factors, pathophysiology, findings, and treatment.
- To highlight the role of advanced imaging in early detection and understanding.
Main Methods:
- Review of current literature on sickle retinopathy in pediatric sickle cell disease.
- Discussion of advanced imaging modalities: ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography (OCTA).
Main Results:
- Advanced imaging offers more sensitive retinal examination, especially in the periphery where sickle retinopathy often first appears.
- OCTA provides noninvasive, quantifiable views of retinal vascular layers.
- Ultra-widefield fluorescein angiography enhances peripheral retinal visualization.
Conclusions:
- Newer imaging may detect a higher prevalence of early sickle retinopathy in children than previously reported.
- Earlier detection can improve understanding of pathogenesis and guide future screening and treatment strategies.
Abstract:
Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Optical coherence tomography angiography provides a noninvasive view of retinal vascular layers that could previously not be imaged and can be quantified for comparative or prospective analyses. Ultra-widefield fluorescein angiography provides a more comprehensive view of the peripheral retina than traditional imaging techniques. Screening for retinopathy by standard fundoscopic imaging modalities detects a prevalence of approximately 10%. In contrast, these more sensitive methods allow for more sensitive examination that includes the retina perimeter where sickle retinopathy is often first detectable. Use of these new imaging modalities may detect a higher prevalence of early sickle pathology among children than has previously been reported. Earlier detection may help in better understanding the pathogenesis of sickle retinopathy and guide future screening and treatment paradigms.
