Pediatric sickle cell retinopathy: Correlation with clinical factors

Rosenberg, Jamie B.; Hutcheson, Kelly A.

doi:10.1016/j.jaapos.2010.11.014

Cited by 42 publications

(42 citation statements)

References 26 publications

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“…One common factor, G6PD deficiency, has been identified as a risk factor, but not at a level of statistical significance (OR 4.20, p=0.054). [17] Overall, more serious disease phenotype, such as previous multiple pain crises or lower hemoglobin or fetal hemoglobin (HbF) levels have been associated with higher prevalence of PSR. [2, 17] To summarize, clinical and hematological factors that have been associated with PSR in HbSS patients are: older age (OR 1.12), longer disease duration (p=0.00), splenectomy (p=0.004), splenic sequestration (OR 4.00), pain crisis (OR 5.00), male sex (OR 2.58-4.20), acute pyelonephritis (OR 2.81), low fetal hemoglobin (p<0.001), and low weight (p<0.05).…”

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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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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“…Most studies assessing sickle retinopathy in pediatric populations utilize FA only when indicated on clinical exam skewing results towards a lower prevalence of retinopathy than in fact exists. 10–12, 23, 24 One retrospective analysis of 258 adolescents with SCD aged 10–18 conducted over a 10 year period found a 20.9% prevalence of SR, 11 while another analysis in children with SCD aged 1 to 18 found an 18.7% prevalence of any retinopathy. 12 Our findings of Goldberg Stage I or II retinopathy in the eyes of all adolescents with SCD aged 10–19 are more consistent with Talbot et al which utilized FA studies in all patients and demonstrated peripheral arteriolar closure (Goldberg Stage I) in 90% of HbSS and HbSC adolescents by age 12.…”

Section: Discussionmentioning

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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“…Although the mechanism was not clear, few studies [19][20][21] reported male sex as a risk factor for developing retinopathy. However, our cohort did not show any sex predominance for retinal changes or PR.…”

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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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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Pediatric sickle cell retinopathy: Correlation with clinical factors

Cited by 42 publications

References 26 publications

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

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

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

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

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