Electroretinogram Findings in Early-Stage Sickle Cell Retinopathy According to Hemoglobin Type

Bottin, Caroline; Racine, Julie; Robert, Matthieu P.; Cohen, Salomon-Yves; Merle, Bénédicte M. J.; Jung, Camille; Mière, Alexandra; Caillaux, Violaine; Souied, Eric H.; Zambrowski, Olivia

doi:10.1167/iovs.16-20719

Cited by 5 publications

(4 citation statements)

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“…Bilateral macular occlusive events may present with binasal field defects [15]. Electrophysiology indicates a selective early involvement of the inner retina in HbSS patients, which may be compatible with vascular occlusions in the superficial and deep capillary plexi in the inner retina [16].…”

Section: Non-proliferative Sickle Cell Retinopathy (Stages I-ii)mentioning

confidence: 99%

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Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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Purpose To provide a focused review of sickle cell retinopathy in the light of recent advances in the pathogenesis, multimodal retinal imaging, management of the condition, and migration trends, which may lead to increased prevalence of the condition in the Western world. Methods Non-systematic focused literature review. Results Sickle retinopathy results from aggregation of abnormal hemoglobin in the red blood cells in the retinal microcirculation, leading to reduced deformability of the red blood cells, stagnant blood flow in the retinal precapillary arterioles, thrombosis, and ischemia. This may be precipitated by hypoxia, acidosis, and hyperosmolarity. Sickle retinopathy may result in sight threatening complications, such as paracentral middle maculopathy or sequelae of proliferative retinopathy, such as vitreous hemorrhage and retinal detachment. New imaging modalities, such as wide-field imaging and optical coherence tomography angiography, have revealed the microstructural features of sickle retinopathy, enabling earlier diagnosis. The vascular growth factor ANGPTL-4 has recently been identified as a potential mediator of progression to proliferative retinopathy and may represent a possible therapeutic target. Laser therapy should be considered for proliferative retinopathy in order to prevent visual loss; however, the evidence is not very strong. With recent development of wide-field imaging, targeted laser to ischemic retina may prove to be beneficial. Exact control of intraoperative intraocular pressure, including valved trocar vitrectomy systems, may improve the outcomes of vitreoretinal surgery for complications, such as vitreous hemorrhage and retinal detachment. Stem cell transplantation and gene therapy are potentially curative treatments, which may prevent retinopathy. Conclusions There is lack of evidence regarding the optimal management of sickle retinopathy. Further study is needed to determine if recent progress in the understanding of the pathophysiology and diagnosis of sickle retinopathy may translate into improved management and outcome.

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Section: Non-proliferative Sickle Cell Retinopathy (Stages I-ii)mentioning

confidence: 99%

“…1). These structures have a high propensity to regress (20-60%) by autoinfarction [11,16]. However, they may also lead to VH or TRD.…”

Section: Proliferative Sickle Cell Retinopathy (Stages Iii-v)mentioning

confidence: 99%

Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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“…Several authors have reported global ERG alterations at the early stages of SCD retinopathy (Bottin et al, 2017;Jung, Chen, Frambach, Rofagha, & Lee, 2016;Peachey et al, 1987). Our study is the first to explore the electrophysiological function of the macula in SCD eyes.…”

Section: Discussionmentioning

confidence: 76%

Multifocal electroretinogram findings in sickle cell maculopathy

Beral

Romana

Lemonne

et al. 2019

Eye

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BACKGROUND: The aim of the present work was to describe and compare multifocal electroretinogram findings (mfERG) in patients with sickle cell disease (SCD) without clinical sign of maculopathy according to the hemoglobin genotype. METHODS: HbSS (homozygous SCD), HbSC (Hemoglobin SC disease) and HbAA (controls) individuals underwent a full ophthalmologic examination (with a fundoscopy), a spectral domain ocular coherence tomography (SD-OCT) and a mfERG. RESULTS: A total of 86 subjects were included: 54 SCD patients (107 eyes) with 32 HbSS (63 eyes) and 22 HbSC (44 eyes) and 32 controls (64 eyes). None of the eyes showed retinal clinical abnormalities. SD-OCT analysis showed that macular thickness was statistically lower in SCD eyes than in controls. mfERG analysis demonstrated a significant reduction of N1 (initial negative deflection), P1 (positive peak) and N2 (second negative deflection) response amplitude densities of HbSS eyes compared to HbAA eyes from the center (<2°) to the periphery (>15°). Implicit time response was also reduced in the center (<2°). N1 and P1 response amplitude densities of HbSC eyes were significantly lower than those of HbAA eyes from the center (<2°) to the periphery (>15°). N2 response amplitude densities were also significantly reduced in the center (<2°) and in the periphery (>10°). N1 implicit time was statistically reduced in HbSC compared to HbSS eyes. CONCLUSION: Our study is the first one to describe macular electrophysiological dysfunction in SCD patients. Moreover, we confirm that SCD maculopathy is equally frequent in HbSS and HbSC genotypes.

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“…Bu çalışma sonucunda hastalık sürecinde HbSS'lilerde iç retinal hücrelerde; HbSC'lilerde ise dış retinal hücrelerde erken tutulum olduğu ileri sürülmüştür. [33] Ayırıcı Tanı OHR'nin ayırıcı tanısında SRAO, santral veya retinal ven dal oklüzyonu, diyabetik retinopati, anemi retinopatisi, hipertansif retinopati, oküler iskemik sendrom, dengue ateşi, radyasyon retinopatisi, lösemi retinopatisi, hiperviskosite retinopatisi, premature retinopatisi, familiyal eksudatif vitreoretinopati, Eales Hastalığı, kronik myeloid lösemi, sarkoidoz, talk retinopatisi, retinal kanama ve NV yapan tüm durumlar göz önüne alınmalıdır.…”

Section: Resim 1 Orak Hücreli Retinopati-renkli Fundus Fotografisiunclassified

Retinal Vascular Findings in Sickle Cell Retinopathy

2023

Güncel Retina (Current Retina)

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Sickle cell disease (SCD) is a common inherited blood disease that develops due to hemoglobin (Hb) dot mutation that reduces the oxygen-carrying ability of erythrocytes. In hypoxia, this mutated abnormal Hb causes "sickling" of erythrocytes, and subsequent episodes of hypoxia, acidosis, inflammation, vaso-occlusive crises, and hemolysis cause progressive damage to many organs, including the brain, kidneys, lungs, bones, heart, and eyes. Systemic and ocular findings vary in SCD. Sickle Cell C Disease and Sickle beta-Thalassemia forms of SCD have mild systemic diseases; however, both cause severe ocular diseases. In the posterior segment of the eye, SCD can cause optic neuropathy, retinopathy, maculopathy, and choroidopathy. Retinal vascular findings include tortuosity in retinal vessels, “silver wire” arterioles, angioid streaks, arterial and venous occlusions, arteriovenous anastomoses, and retinal neovascularizations (NVs). Fundus fluorescein angiography (FFA), optical coherence tomography (OCT), and OCT angiography are used in the diagnosis. NVs and the enlargement and irregularity of foveal avascular zone (FAZ) in FFA, macular temporal retinal thinning in OCT, FAZ enlargement, and the reduction in densities of macular capillary in OCT angiography are the main findings. This article is aimed to review the retinal vascular findings encountered in Sickle Cell Retinopathy.

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Electroretinogram Findings in Early-Stage Sickle Cell Retinopathy According to Hemoglobin Type

Abstract: These results could suggest an early involvement of the inner retinal cells in the disease process in HbSS patients and of the outer retinal cells in HbSC patients. This could provide new insights on the pathophysiology of the retinal affection in HbSS/HbSC SC disease.

Cited by 5 publications

References 28 publications

Sickle cell retinopathy. A focused review

Sickle cell retinopathy. A focused review

Multifocal electroretinogram findings in sickle cell maculopathy

Retinal Vascular Findings in Sickle Cell Retinopathy

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