Ocular manifestations of sickle cell disease

Fadugbagbe, A. O.; Gurgel, Ricardo Queiróz; Mendonça, C. Q.; Cipolotti, Rosana; Santos, Allisson Mário dos; Cuevas, Luís E.

doi:10.1179/146532810x12637745451870

Cited by 42 publications

(44 citation statements)

References 56 publications

(65 reference statements)

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“…Several studies reported an increase in incidence and prevalence rates of all ocular manifestations with increasing age [3,19]. In our cohort, all retinal changes were detected above the age of 12 years and the youngest patient showing PR was 15 years old.…”

Section: Discussionsupporting

confidence: 49%

“…Increase in life expectancy of SCD patients in recent years has led to emergence of more disease-related complications, including ocular manifestations [3]. SCD can affect every vascular bed in the eye and can cause blindness in advanced stages [4].…”

Section: Introductionmentioning

confidence: 99%

“…Vaso-occlusive disease of the retina can be responsible for nonproliferative and proliferative ocular changes (based on the absence or presence of vascular proliferation respectively). This distinction is important because formation of new vessels is the single most important precursor of potentially blinding complication [3].…”

Section: Introductionmentioning

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: 49%

Section: Introductionmentioning

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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“…As manifestações oculares da anemia falciforme compreendem alterações orbitárias, conjuntivais, uveais, papilares e, principalmente, retinianas (7)(8)(9)(10) . A retina periférica e a mácula parecem ser os locais mais suscetíveis à oclusão vascular, onde as lesões são mais evidentes e destrutivas, podendo ocasionar cegueira (11)(12)(13) .…”

Section: Alterações Retinianas Em Jovens Portadores De Anemia Falcifounclassified

Alterações retinianas em jovens portadores de anemia falciforme (hemoglobinopatias) em hospital universitário no nordeste do Brasil

Santos¹,

Faro²,

Amaral³

et al. 2012

Arq. Bras. Oftalmol.

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RESUMOObjetivos: Descrever e classificar alterações retinianas encontradas em portadores de anemia falciforme com genótipo SS, bem com comparar métodos diagnósticos (mapeamento de retina e angiofluoresceinografia). Métodos: Neste estudo transversal foram avaliados pacientes portadores de anemia falciforme com idade igual ou superior a sete anos. Esses pacientes foram submetidos a mapeamento de retina e angiofluoresceinografia. Os achados do mapeamento de retina foram agrupados em três classes: sem alterações; alterações não proliferativas e alterações proliferativas. Os resultados à angiofluoresceinografia foram classificados de acordo com os estágios de Goldberg, variando de I a V e expressando gradiente crescente de gravidade. Resultados: Foram avaliados 61olhos de 31pacientes. A retinopatia falciforme foi encontrada em 38/61 (62,3%) dos olhos examinados. A média de idade do grupo de portadores de retinopatia foi menor que dos pacientes sem retinopatia (14,4 versus 17,4 anos, p=0,04). Observou-se elevada freqüência de retinopatia não proliferativa, especialmente as tortuosidades vasculares (27,9%), seguidas por anastomoses arteriovenosas na periferia da retina (24,6%) e oclusões arteriolares (8,2%). Em um olho foi observado neovascularização. Em 16,4% dos olhos obteve-se resultado normal no mapeamento de retina e alterado à angiofluoresceinografia. Conclusões: As alterações retinianas do tipo não proliferativa são frequentes e precoces nos portadores de anemia falciforme do tipo SS, sendo a angiofluoresceinografia mais sensível no diagnóstico quando comparada ao mapeamento de retina. Descritores INTRODUÇÃOA anemia falciforme é a doença hereditária mais prevalente no Brasil (1) . A hemoglobinopatia ocorre em situação de homozigose do gene responsável pela produção de uma hemoglobina mutante, a hemoglobina S, que em seu estado desoxigenado pode sofrer polimerização e originar hemácias falcizadas, responsáveis por anemia hemolítica crônica e fenômenos vasoclusivos (1)(2)(3)(4)(5)(6) .Embora haja outros genótipos para a doença falciforme, acredita-se que SC, Sβ 0 talassemia, em menor grau, o SS (este o responsável pelo Alterações retinianas em jovens portadores de anemia falciforme (hemoglobinopatias) em hospital universitário no nordeste do BrasilRetinal impairment in young individuals with sickle cell anemia (hemoglobin ss disease) in university hospital in Northeastern of Brazil

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“…Sickle cell retinopathy (SCR) results from the microvascular occlusions induced by sickle-shaped erythrocytes in the retina [4]. It is considered as the major ocular manifestation of SCD to produce visual impairment and blindness [5]. The anatomical changes in the retina are highly associated with the disease progression.…”

Section: Introductionmentioning

confidence: 99%

Computer-aided classification of sickle cell retinopathy using quantitative features in optical coherence tomography angiography

Alam

Thapa

Lim

et al. 2017

Biomed. Opt. Express

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As a new optical coherence tomography (OCT) imaging modality, there is no standardized quantitative interpretation of OCT angiography (OCTA) characteristics of sickle cell retinopathy (SCR). This study is to demonstrate computer-aided SCR classification using quantitative OCTA features, i.e., blood vessel tortuosity (BVT), blood vessel diameter (BVD), vessel perimeter index (VPI), foveal avascular zone (FAZ) area, FAZ contour irregularity, parafoveal avascular density (PAD). It was observed that combined features show improved classification performance, compared to single feature. Three classifiers, including support vector machine (SVM), k-nearest neighbor (KNN) algorithm, and discriminant analysis, were evaluated. Sensitivity, specificity, and accuracy were quantified to assess the performance of each classifier. For SCR vs. control classification, all three classifiers performed well with an average accuracy of 95% using the six quantitative OCTA features. For mild vs. severe stage retinopathy classification, SVM shows better (97% accuracy) performance, compared to KNN algorithm (95% accuracy) and discriminant analysis (88% accuracy). "Quantitative assessment of the retinal microvasculature using optical coherence tomography angiography," J.

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Ocular manifestations of sickle cell disease

Cited by 42 publications

References 56 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

Alterações retinianas em jovens portadores de anemia falciforme (hemoglobinopatias) em hospital universitário no nordeste do Brasil

Computer-aided classification of sickle cell retinopathy using quantitative features in optical coherence tomography angiography

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