Retinal changes in children and adolescents with sickle cell disease attending a paediatric hospital in Cairo, Egypt: risk factors and relation to ophthalmic and cerebral blood flow

Tantawy, Azza Abdel Gawad; Andrawes, Nevine G.; Adly, Amira Abdel Moneam; Kady, Bassam A. El; Shalash, Ali

doi:10.1093/trstmh/trt008

Cited by 17 publications

(20 citation statements)

References 22 publications

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“…Absence of other non-proliferative ocular signs as salmon patches and iridescent spots among our patients, as previously reported [17], may be attributed to their transient status. Our study failed to detect any proliferative retinal lesions beyond stage I confirming the low frequency of such changes in Egyptian cohort [18].…”

Section: Discussionsupporting

confidence: 64%

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Ocular Manifestations in Egyptian Children and Young Adults with Sickle Cell Disease

El-Ghamrawy

Behairy

Menshawy

et al. 2014

Indian J Hematol Blood Transfus

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In sickle cell disease (SCD), ocular lesions result from stasis and occlusion of small eye vessels by sickled erythrocytes. Vaso-occlusive disease of the retina can be responsible for nonproliferative (NPR) and proliferative retinopathy (PR). Patients are often asymptomatic until serious complications arise as, vitreous hemorrhage and retinal detachment. This work aimed to study the frequency and pattern of ocular manifestations in Egyptian children and young adults with SCD. In this cross-sectional study, 40 steady state patients (80 eyes) aged 2-28 years (30 children and 10 young adults) with established diagnosis of SCD (26 with homozygous SS and 14 with S/b thalassemia underwent complete ophthalmic examination with dilated fundoscopy. Fluorescein angiography was performed for patients C12 years old. The overall frequency of retinal lesions was 47.5 % (46.2 and 50 % of SS and S/b patients respectively). PR and NPR were evident in 32.5 and 27.5 % of all enrolled patients respectively (five patients having both). Peripheral retinal occlusion was a frequent ocular finding in both groups; the youngest patient showing PR was 15 years old. Older age, longer disease duration and splenectomy were significantly more prevalent among patients with PR. Despite lack of visual symptoms, children and young adults are at risk of PR. Frequency of retinal lesions was comparable in SS and S/b patients. Periodic ophthalmologic examination starting at the age of 12 years is recommended for timely-identification of retinal lesions thus minimizing the risk of sight threatening retinopathy.

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Section: Discussionsupporting

confidence: 64%

“…The hematological correlates with retinal lesions are still unclear. Conflicting reports suggest possible effects of total hemoglobin, fetal hemoglobin and HbS [18,[21][22][23].…”

Section: Discussionmentioning

confidence: 99%

Ocular Manifestations in Egyptian Children and Young Adults with Sickle Cell Disease

El-Ghamrawy

Behairy

Menshawy

et al. 2014

Indian J Hematol Blood Transfus

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“…By standard dilated fundoscopic examination of pediatric patients, the prevalence of retinal vascular pathology has been estimated to be anywhere between 17% and 94%. [78, 79] Most of the pathology found reflects early vascular changes rather than the more serious injury that can progress to visual loss found in adult patients. Future studies could include the prevalence, progression, clinical outcomes and clinical utility of the newer diagnostic imaging techniques such as SD-OCT, OCT-A, and UWFA and the impact of new early screening and therapeutic approaches on eye health in pediatric SCD.…”

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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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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“…In the posterior segment, manifestations include retinal hemorrhage and exudate, as well as angioid streaks, chorioretinal infarction, vitreous hemorrhage, occlusion of the central retinal artery or its branches, and proliferative retinopathy. Arteriolar occlusion and loss of capillary perfusion in the periphery of the retina are the most striking features of sickle cell retinopathy, being more common in HbSC hemoglobinopathy than in the HbSS form and occurring predominantly in the superior temporal region; ischemic areas release substances that stimulate angiogenesis, and the initial vascular remodeling at the junction between the nonperfused periphery and the perfused central region promotes the formation of arteriovenous anastomoses (8,17) .…”

Section: Discussionmentioning

confidence: 99%

“…The formation of new blood vessels is the most important factor that can lead to amaurotic complications that precede the occurrence of vitreous hemorrhage or retinal detachment. Asymptomatic ocular conditions may develop, regardless of the progression of the disease, and can have devastating consequences (8) .…”

Section: Introductionmentioning

confidence: 99%

Doppler velocimetry of the orbital arteries in patients with sickle cell anemia: relationship with biomarkers of hemolysis

et al. 2017

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ObjectiveTo investigate orbital vascular resistance by Doppler velocimetry in patients with steady-state sickle cell anemia, as well as to characterize its relationship with biomarkers of hemolysis.Materials and MethodsThis was a cross-sectional study of two groups: 71 outpatients with sickle cell anemia; and 32 age- and gendermatched, healthy subjects (control group). All participants underwent Doppler velocimetry of the orbital arteries and laboratory tests.ResultsAll biochemical laboratory markers were abnormal in the sickle cell anemia patients (p < 0.0001 vs. controls). In the patient group, Doppler velocimetry revealed increased flow velocities in the ophthalmic artery and reduced flow velocities in the central retinal artery, as well as high values for the resistance index (RI) and pulsatility index (PI) in both arteries (p < 0.0001 vs. controls). Biomarkers of hemolysis were found to correlate significantly with the RI and PI indices. In the ophthalmic artery, the reticulocyte count showed a moderate direct correlation with RI and with PI. In the central retinal artery, hemoglobin showed a strong inverse correlation with RI and with PI.ConclusionOrbital vascular resistance, as evaluated by Doppler velocimetry, is elevated in patients with steady-state sickle cell anemia and shows a significant correlation with biomarkers of hemolysis.

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Retinal changes in children and adolescents with sickle cell disease attending a paediatric hospital in Cairo, Egypt: risk factors and relation to ophthalmic and cerebral blood flow

Abstract: Sickle retinopathy was correlated with TAMV in MCAs but not in OAs. A significant difference was found between initial and follow-up TAMVs in the MCAs, after 1 year of regular HU and transfusion therapy, in those with conditional velocities.

Cited by 17 publications

References 22 publications

Ocular Manifestations in Egyptian Children and Young Adults with Sickle Cell Disease

Ocular Manifestations in Egyptian Children and Young Adults with Sickle Cell Disease

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

Doppler velocimetry of the orbital arteries in patients with sickle cell anemia: relationship with biomarkers of hemolysis

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