Optical Coherence Tomography Angiography of Sickle Cell Maculopathy

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 non-invasive 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.

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“…[44-51] Some of these silent findings have been shown to be significantly associated with the presence of PSR. [49]…”

Section: Resultsmentioning

confidence: 99%

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

Pahl

Green²,

Bhatia³

et al. 2017

Journal of Pediatric Hematology/Oncology

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“…The nonperfusion of capillaries in the deep layer seen on OCTA has been reported in various retinal diseases. 6,20,[39][40][41][42] When compared with the superficial plexus, the deep capillaries may be more prone to ischemia because they are positioned in a watershed-like region and are not directly connected to arterioles. 43,44 The major blood vessels are found in the thickest portions of the RNFL, which are located in the superior and inferior regions.…”

Section: Discussionmentioning

confidence: 99%

Inner Retinal Microvasculature Damage Correlates With Outer Retinal Disruption During Remission in Behçet's Posterior Uveitis by Optical Coherence Tomography Angiography

Cheng

Shen

Zhuang

et al. 2018

Invest. Ophthalmol. Vis. Sci.

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“…13–17 Recent reports have shown that UWFA, which captures up to twice as much retinal area as conventional fluorescein angiography (FA) 18 , is more sensitive than traditional FA in identifying peripheral vascular changes in sickle retinopathy. 14 Additionally, proliferative sickle retinopathy has been found to be more prevalent in patients with macular thinning identified on SD-OCT suggesting the utility of SD-OCT in identifying patients at higher risk for proliferative disease.…”

Section: Introductionmentioning

confidence: 99%

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy

Pahl

Green

Bhatia

et al. 2017

American Journal of Ophthalmology

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Purpose Using standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCT-A) detect more frequent retinopathy in adolescents with SCD. Design Cross-sectional study. Methods Setting Institutional. Subjects Sixteen adolescents with SCD, ages 10–19 years, (mean age 14.9 years) and 5 age-equivalent controls (mean age 17.4 years). Observation Procedures Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT and OCT-A were performed. Main Outcome Measures Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I–V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT. Results While 22/32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4/24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20/24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III–V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6/30 (20%) eyes. Conclusions All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.

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Optical Coherence Tomography Angiography of Sickle Cell Maculopathy

Cited by 35 publications

References 7 publications

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

Inner Retinal Microvasculature Damage Correlates With Outer Retinal Disruption During Remission in Behçet's Posterior Uveitis by Optical Coherence Tomography Angiography

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy

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