Sickle cell retinopathy: What we now understand using optical coherence tomography angiography. A systematic review

Sickle cell retinopathy is often initially asymptomatic even in proliferative stages, but can progress to cause vision loss due to vitreous haemorrhages or tractional retinal detachments. Challenges with access and adherence to screening dilated fundus examinations, particularly in medically underserved areas where the burden of sickle cell disease is highest, highlight the need for novel approaches to screening for patients with vision-threatening sickle cell retinopathy. This article reviews the existing literature on and suggests future research directions for coupling artificial intelligence with multimodal retinal imaging to expand access to automated, accurate, imaging-based screening for sickle cell retinopathy. Given the variability in retinal specialist practice patterns with regards to monitoring and treatment of sickle cell retinopathy, we also discuss recent progress toward development of machine learning models that can quantitatively track disease progression over time. These artificial intelligence-based applications have great potential for informing evidence-based and resource-efficient clinical diagnosis and management of sickle cell retinopathy.

Artificial intelligence for improving sickle cell retinopathy diagnosis and management.

Objectives To examine interocular asymmetry of foveal avascular zone (FAZ) and parafoveal capillary density metrics in sickle cell retinopathy (SCR) using optical coherence tomography angiography (OCT-A). Methods This cross-sectional, retrospective study evaluated SCR patients and unaffected controls who underwent 3x3mm macular OCT-A imaging using a spectral domain-OCT system. FAZ (area, perimeter, and acircularity index) and parafoveal capillary density metrics were computed for both eyes of each participant. In unaffected controls, interocular difference in FAZ and parafoveal capillary density metrics were evaluated using Bland-Altman plots. SCR patients with interocular difference outside the upper 97.5% and lower 2.5% limits of agreement from controls were defined as having interocular asymmetry. Area under receiver operating characteristic curve (AROC) was also performed to determine the ability of the absolute interocular difference to differentiate between subjects with SCR—including non-proliferative SCR (NP-SCR) and proliferative SCR (P-SCR)–and unaffected controls. Results Thirty-one patients with SCR (21 NP-SCR and 10 P-SCR) and 14 race-matched and age-matched controls were included for analysis. Interocular asymmetry was seen for all FAZ and parafoveal capillary density metrics in NP-SCR and P-SCR subjects. SCR subjects showed greater disease severity in the left-eye for FAZ and parafoveal capillary density metrics. Conclusions NP-SCR and P-SCR patients demonstrated quantifiable interocular asymmetry in FAZ and parafoveal capillary density metrics compared to unaffected subjects, with left-eye predominance in disease severity.

/pdf/interocular-asymmetry-of-foveal-avascular-zone-morphology-3f1ddg108u.pdf

Interocular asymmetry of foveal avascular zone morphology and parafoveal capillary density in sickle cell retinopathy.

OBJECTIVE The aim of this study was to compare optical coherence tomography (OCT) findings in pediatric patients with sickle cell disease (SCD) and healthy individuals and to investigate associations between these data and the patients' systemic findings. MATERIALS AND METHODS The study included 108 eyes of 54 patients with SCD with no visual symptoms and a control group consisting of 110 eyes of 55 healthy subjects with no systemic or ocular pathology. After best-corrected visual acuity assessment, the study participants underwent a complete ophthalmologic examination including intraocular pressure. After examination and pupil dilation induced with 1% tropicamide, 9×9 mm macular sections were obtained with spectral-domain OCT. The macular sections were evaluated according to Early Treatment Diabetic Retinopathy Study (ETDRS) map and internal and external retinal thicknesses were measured using the software included in the OCT device. RESULTS The patient group showed significantly more foveal flattening, temporal thinning, and vascular tortuosity than the control group (P<0.0001 for all). Foveal width was significantly greater in the patient group (1592.39±175.56 µm) compared with the control group (1391.01±175.56 µm) (P<0.0001), whereas foveal depth was significantly lower in the patient group (121.15±26.83 µm) than in the control group (146.1±12.25 µm) (P<0.0001). The mean total retinal thickness was 253.53±22.31 µm in the patient group and 261.03±18.48 µm in the control group (P=0.007). Similarly, central retinal thickness was significantly lower in the patient group (219.35±10.53 µm) compared with the control group (235.32±12.51 µm) (P<0.0001). DISCUSSION Our study shows that pediatric patients with SCD may have subclinical retinal involvement and that temporal thinning, in particular, is an important OCT finding. This strongly suggests that OCT imaging would be a beneficial addition to routine ophthalmologic examination in the diagnosis and follow-up of this patient group.

Evaluation of Ocular Complications by Using Optical Coherence Tomography in Children With Sickle Cell Disease Eye Findings in Children With Sickle Cell Disease.

Optical coherence tomography is an increasingly important part of the retinal specialist's and general ophthalmologist's toolkit for diagnosing and managing retinal disease. This review summarizes the unique considerations and available imaging systems with which pediatric ophthalmologists should be familiar when attempting optical coherence tomography in children. Normal developmental changes in foveal and extrafoveal structure and the need for an established pediatric normative database of retinal thicknesses are reviewed. Finally, applications of optical coherence tomography imaging to selected representative pediatric retinal diseases are introduced as examples of how optical coherence tomography in children is furthering the diagnosis and management of vision-threatening retinal diseases.

Optical coherence tomography imaging of the pediatric retina

Purpose To compare the results of fundus examination and spectral domain optic coherence tomography (SD-OCT) in detecting retinal changes in pediatric patients with sickle cell disease at a single center. Methods In this prospective study, conducted over a period of 19 months, consecutive African American patients with sickle cell disease underwent complete ophthalmologic examination, and SD-OCT images of the maculas of both eyes were obtained. Results A total of 69 (37 males) patients aged 5-20 years (mean 12.89 ± 4.09; range, 2-20) with sickle cell disease (SC, 26; SS, 36; Sβ+, 5; Sβ0 thalassemia, 2) were examined. Patients’ visual acuity range was 20/20 to 20/40. On funduscopic examination, 11 of 69 showed signs of retinopathy, whereas 47 of 68 showed inner retina thinning in the watershed zone temporal to the fovea on SD-OCT. On average, SD-OCT diagnosed disease 1.78 years earlier than fundus examination. Of patients Conclusions The peripheral retina could be visualized on fundus examination but not easily imaged on SD-OCT, which, however, had a higher detection rate and offered earlier diagnosis. In our patient cohort SD-OCT showed that the severity of retinal change was associated with more severe sickle cell disease genotypes (SS and Sβ0).

Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.

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Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.