Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors

Grego, Lisa; Pignatto, Silvia; Alfier, F; Arigliani, Michele; Rizzetto, Francesca; Rassu, Nicolò; Samassa, Francesco; Prosperi, Raffaele; Barbieri, F; Dall'Amico, R; Cogo, Paola; Lanzetta, Paolo

doi:10.1007/s00417-020-04764-y

Cited by 10 publications

(13 citation statements)

References 28 publications

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“…OCTA of thinned macular areas reveals flow void affections in the superficial and deep vascular plexuses in both children and adults, albeit the areas of flow void affection are more widespread in the deep arteries due to greater oxygen demand [ 24 , 25 ]. In our study, the flow void significantly involved the deep vascular plexus, with insignificant affection of the superficial plexus in agreement with Grego et al [ 21 ] who concluded that sickle cell maculopathy can progress, first affecting the flow voids in the deep vascular plexus and then the superficial plexus.…”

Section: Discussionsupporting

confidence: 92%

“…In our investigation, OCT and OCTA detected SCM as macular thinning and affection of flow voids in the deep vascular plexus, respectively, in 10 eyes of 7 patients, 9 eyes with Hb SS children, and 1 eye with a child with Hb SC. In agreement with Grego et al [ 21 ], early prediction of SCR and SCM was detected in children by SD-OCT and OCTA. These two retinal problems were more prevalent in children with a history of hemolysis.…”

Section: Discussionsupporting

confidence: 91%

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The Impact of Optical Coherence Tomography in the Early Identification of Children with Sickle Cell Retinopathy

Abdelkader

Shaaban

Zahran

et al. 2022

International Journal of Clinical Practice

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Introduction. Sickle cell disease is characterized by the production of abnormal hemoglobin, which affects hemoglobin molecule stability during hypoxia and leads to the formation of sickle cells, resulting in increased hematic viscosity, hemolysis, and microvascular blockage. Vasoocclusion is assumed to be the primary cause of vision-threatening retinopathy in sickle cell disease. The aim of this study was to improve the early detection of sickle cell retinopathy (SCR) in children with sickle cell disease (SCD) and investigate the link between systemic and ocular symptoms. Methods. This cross-sectional study comprised children with SCD. The patient files provided a detailed medical history. The laboratory tests included a blood count, reticulocyte count, and Hb electrophoresis. The slit lamp, fundus, optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) were all part of the ophthalmological examination. Results. The study comprised 15 children with sickle cell disease who met the inclusion and exclusion criteria, with a mean age of 11.15 ± 1.29 years. Nine of the children were males (60%) and six were females (40%). 8 (53.3%) of the children had Hb SS, three (20%) had Hb SC, three (20%) had Hb SB+, and one (6.7%) had Hb SB0. Four children (26.7%) had poor visual acuity. A fundus examination revealed significant abnormal findings in 12 of the 7 children’s eyes (40 percent). Macular thinning was detected by OCT in 10 eyes of 7 children (33.3%). Flow voids at the deep retinal capillary plexus were detected by OCTA in 10 eyes of 7 children (33.3%). Longer disease duration, higher reticulocytic percent, more painful crises, and noncompliance with hydroxyurea medication were all linked to the existence of eye abnormalities on fundus examination and OCT. Conclusion. OCTA can show early retinal damage in sickle cell patients with macular changes. Sickle cell retinopathy is usually associated with more severe disease.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 91%

The Impact of Optical Coherence Tomography in the Early Identification of Children with Sickle Cell Retinopathy

Abdelkader

Shaaban

Zahran

et al. 2022

International Journal of Clinical Practice

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“…However, studies have revealed pathologies correlated with SD-OCT in the perfusion defect areas detected by OCTA. 21,22 Grego et al 22 did not detect additional flow gap areas other than macular thinning identified by SD-OCT in OCTA, and stated that the SD-OCT findings supported the OCTA findings in patients with SCA.…”

Section: Discussionmentioning

confidence: 93%

“…Grego et al 22 reported that complications such as retinopathy and maculopathy are associated with hemolysis indices such as low hemoglobin and hematocrit rates, high reticulocyte percentage, and high total bilirubin levels. Vatansever et al 25 stated that there was no difference in foveal flattening and temporal thinning between with and without a history of sequestration crisis in patients with SCA.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of retinal nerve fiber layer and choroidal thickness with spectral domain optical coherence tomography in children with sickle cell anemia

et al. 2021

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Background. The aim of this study is to examine the thickness of choroidal, macular and peripapillary retinal nerve fiber layer by spectral-domain optical coherence tomography (SD-OCT) in pediatric patients with sickle cell anemia (SCA) without retinopathy. Methods. A total of 75 children (30 SCA patients (Group 1) and 45 healthy individuals (Group 2) were included in the study. Macular (central, superior, inferior, nasal, temporal), choroidal (subfoveal, at nasal distances from the central fovea of 1000 μm [N1], 2000 μm [N2], 3000 μm [N3], at temporal distances from the central fovea of 1000 μm [T1], 2000 μm [T2], 3000 μm [T3]) and RNFL (average, temporal, superotemporal, inferotemporal, nasal, inferonasal and superonasal) measurements were performed by SD-OCT. These parameters were compared with healthy children with similar demographic characteristics. Results. The mean age was 14.11±3.86 (11-18) in sickle cell anemia patients and 13.15± 2.69 (10-18) in the healthy control group. Of the patients, 56.6% (n=17) of Group 1 and 44.4% (n=20) of Group 2 were male. Choroidal measurements made in the subfoveal, N1, N2, N3, T1, T2 and T3 quadrants showed that the choroid was thinner in 6 quadrants in SCA patients compared to the healthy group (p = 0.003, p = 0.039, p = 0.035, p = 0.595, p = 0.006, p = 0.005, p = 0.047, respectively). In RNFL measurements, there was significant thinning in the temporal, inferotemporal, and nasal quadrants of SCA patients compared to the healthy group. Changes in other quadrants were not significant. Conclusions. SD-OCT is a useful imaging method in the diagnosis and screening in patients with SCA without retinopathy. Early diagnosis of retinopathy during subclinical disease will prevent visual loss in these patients.

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“…33 Most recently, a prospective assessment of OCTA images in pediatric patients with sickle cell disease showed flow voids in areas of retinal thinning in all included individuals. 34 It was observed that in all the patients older than 10 years with the genotype HbSS, the flow voids involved both plexuses with more prominent voids in the DCP.…”

Section: Sickle Cell Retinopathymentioning

confidence: 89%

Optical Coherence Tomography Angiography in Pediatric Retinal Disorders

Ponugoti

Baumal

Vajzovic

2022

Journal of VitreoRetinal Diseases

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Purpose: The rapid and noninvasive nature of optical coherence tomography angiography (OCTA) makes it a potentially valuable tool for imaging the retina in children. With the optimization of tabletop systems and the development of experimental handheld OCTA devices, there is expanded potential for OCTA in the clinic and the operating room. This article reviews the utility of OCTA in some of the most common pediatric retinal disorders. Methods: A thorough computerized PubMed search was performed to review relevant published journal articles to contextualize and identify the role of OCTA in common retinal disorders with vascular involvement affecting children. Pertinent results and findings from original investigations and case reports were summarized. Results: The ability to quickly collect both qualitative and quantitative information about retinal microvasculature, in both the clinic and operating room settings, with OCTA, has led to the uncovering of microvascular features and morphologic changes in many pediatric retinal disorders such as Coats Disease, familial exudative vitreoretinopathy, incontinentia pigmenti, sickle cell retinopathy, Stargardt Disease, X-linked juvenile retinoschisis, retinopathy of prematurity, diabetic retinopathy in type 1 diabetes, pediatric retinal tumors, and choroidal neovascularization. Conclusions: OCTA is a relevant tool to aid early detection, guide intervention, monitor treatment response, and understand pathogenesis in a number of pediatric retinal disorders.

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Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors

Cited by 10 publications

References 28 publications

The Impact of Optical Coherence Tomography in the Early Identification of Children with Sickle Cell Retinopathy

The Impact of Optical Coherence Tomography in the Early Identification of Children with Sickle Cell Retinopathy

Evaluation of retinal nerve fiber layer and choroidal thickness with spectral domain optical coherence tomography in children with sickle cell anemia

Optical Coherence Tomography Angiography in Pediatric Retinal Disorders

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