Sickle cell retinopathy in Jamaican children: further observations from a cohort study.

Talbot, J F; Bird, Alan C.; Maude, G. H.; Acheson, R W; Moriarty, Brendan; Serjeant, G. R.

doi:10.1136/bjo.72.10.727

Cited by 53 publications

(34 citation statements)

References 16 publications

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“…This may be attributed to our smaller sample size. Comparing frequencies of disease-related events and VOE between patients with PR and those without in our cohort revealed no significant differences, which support previous reports [4,21]. However, this conflicts previous observations of ocular complications being attributed directly or indirectly to vaso-occlusion.…”

Section: Discussionsupporting

confidence: 83%

“…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%

“…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%

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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: 83%

Section: Discussionmentioning

confidence: 99%

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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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“…A freqüência de retinopatia foi maior no grupo de faixa etária acima de 10 anos, semelhante a outros estudos (16)(17)(18) , alertando para a importância do exame oftalmológico precoce e periódico em pacientes portadores de hemoglobinopatias.…”

Section: Discussionunclassified

“…Conforme os estudos de Gonçalves et al (10) e Talbot et al (17) , a retinopatia não proliferativa é mais freqüente nos pacientes jovens, enquanto que a forma proliferativa ocorre numa faixa etária mais elevada, acima dos vinte anos de idade. De forma semelhante, não detectamos caso de retinopatia proliferativa, provavelmente pela baixa idade das crianças estudadas.…”

Section: Discussionunclassified

Achados fundoscópicos em crianças portadoras de anemia falciforme no estado do Rio Grande do Norte

Garcia¹,

Fernandes²,

Uchôa³

et al. 2002

Arq. Bras. Oftalmol.

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Objetivos: Detectar os principais achados fundoscópicos em crianças portadoras de hemoglobinopatias falciformes. Métodos: Foram estudados 26 pacientes com hemoglobinopatias falciformes, no Serviço de Oftalmologia do Hospital Universitário Onofre Lopes, Natal, RN, que foram submetidos a protocolo de pesquisa pré-estabelecido. Os resultados foram avaliados estatisticamente pelo teste qui-quadrado. Resultados: A idade média foi de 10,6 anos, com acuidade visual igual ou melhor que 20/25 na maioria, excetuando-se 3 olhos, que apresentavam outras doenças associadas. O tipo mais freqüente foi o SS com 57,7% (15/26) dos casos, seguido pelos SC e SA com 15,4% (4/26) cada, e pelo S-Thal com 11,5% (3/26). A freqüência da retinopatia por células falciformes foi maior após os 10 anos de idade, sendo mais freqüente, em valores relativos, no tipo S-Thal (100% dos casos) e, em valores absolutos, no tipo SS (9 casos). Os dois achados mais comuns foram tortuosidade venosa (12/26) e "black sunburst" (7/26). Conclusões: Observamos que a incidência de retinopatia por células falciformes aumentou após os 10 anos de idade e não evidenciamos achados da doença proliferativa. Portanto, enfatizamos a necessidade do exame oftalmológico precoce nos portadores de anemia falciforme, como forma de prevenir futuras complicações oculares.

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Monitoring retinal pathology and cerebral injury in sickle cell disease using spectral‐domain optical coherence tomography in pediatric patients

Jin

Kandula

Miller

2021

Pediatric Blood & Cancer

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Purpose: This study aimed to confirm the correlation between sickle cell disease (SCD) genotype and retinal damage identified by spectral-domain optical coherence tomography (SD-OCT), and examine a potential link between hypoxic ischemic injury in the retina and brain. Methods: In this prospective, observational case series, 117 patients (56 males) aged 5-20 years with SCD (36 SC, 68 SS, eight Sβ+ thalassemia, five Sβ0 thalassemia) underwent ophthalmologic examination including funduscopy and SD-OCT imaging. Comparison of SCD genotypes and association between ocular findings and cerebrovascular disease (CVD) in subjects with SS/Sβ0 genotype were investigated. Results: Visual acuity ranged from 20/20 to 20/40. On funduscopic exam, 16 of 117 (13.7%) had retinopathy; 69 of 117 (59.0%) showed inner retina thinning on SD-OCT. Patients with SS/Sβ0 showed a higher frequency of sickle cell retinopathy (SCR) change (68.5% vs. 47.2%), bilateral SCR (49.9% vs. 25.0%), and foveal involvement (15.1% vs. 0) than the SC genotype. While funduscopic findings in our cohort with SS/Sβ0 genotype showed no correlation with CVD, 20 of 21 patients with CVD had abnormal SD-OCT. Elevated reticulocyte percentage and aspartate aminotransferase are associated with SD-OCT changes and CVD.Conclusions: SD-OCT was better than funduscopy in detecting retinal changes, higher frequency, and more extensive retinal changes in the more severe SCD genotypes SS and Sβ0 as compared with SC. The correlation between abnormal SD-OCT and CVD strongly suggests that retinal exam using SD-OCT may aid in detection and monitoring SCD-related CVD. Retinopathy may be another component of the hemolytic subphenotype of SCD.

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Sickle cell retinopathy in Jamaican children: further observations from a cohort study.

Cited by 53 publications

References 16 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

Achados fundoscópicos em crianças portadoras de anemia falciforme no estado do Rio Grande do Norte

Monitoring retinal pathology and cerebral injury in sickle cell disease using spectral‐domain optical coherence tomography in pediatric patients

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