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. Non-systematic focused literature review. 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. 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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Sickle cell retinopathy. A focused review.

Sickle cell retinopathy (SCR) is the most representative ophthalmologic complication of sickle cell disease (SCD), a hemoglobinopathy affecting both adults and children. SCR presents a wide spectrum of manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated at the earliest. Over the past decade, multidisciplinary research developments have focused upon systemic, genetic, and ocular risk factors of SCR, enabling the clinician to better diagnose and manage these patients. In addition, newer imaging and testing modalities, such as spectral domain-optical coherence tomography angiography, have resulted in the detection of subclinical retinopathy related to SCD. Innovative therapy includes intravitreal injection of an anti-vascular endothelial growth factor (eg, Lucentis® [ranibizumab] or Eylea® [aflibercept]) which appears comparatively safe and efficient, and may be combined with laser photocoagulation (LPC) for proliferative SCR. The effect of LPC alone does not significantly lead to the regression of advanced SCR, although it helps in avoiding hemorrhage and sight loss. This comprehensive article is based on 10-years retrospective (2007-2017) studies. It aims to present advances and recommendations in SCR theranostics while pointing out the requirement of combinatorial approaches for better management of SCR patients. To reach this goal, we identified and analyzed randomized original and review articles, clinical trials, non-randomized intervention studies, and observational studies using specified keywords in various databases (eg, Medline, Embase, Cochrane, ClinicalTrials.gov).

https://www.dovepress.com/getfile.php?fileID=38184

Sickle cell retinopathy: improving care with a multidisciplinary approach.

Clinical and Ophthalmic Factors Associated With the Severity of Sickle Cell Retinopathy

Wide-field imaging is a newer retinal imaging technology, capturing up to 200 degrees of the retina in a single photograph. Individuals with sickle cell retinopathy commonly exhibit peripheral retinal ischemia. Patients with proliferative sickle cell retinopathy develop pathologic retinal neovascularization of the peripheral retina which may progress into sight-threatening sequelae of vitreous hemorrhage and/or retinal detachment. The purpose of this review is to provide an overview of current and future applications of wide-field retinal imaging for sickle cell retinopathy, and recommend indications for best use. There are several advantages to wide-field imaging in the clinical management of sickle cell disease patients. Retrospective and prospective studies support the success of wide-field imaging in detecting more sickle cell induced retinal microvascular abnormalities than traditional non-wide-field imaging. Clinicians can easily capture a greater extent of the retinal periphery in a patient’s clinical baseline imaging to follow the changes at an earlier point and determine the rate of progression over time. Wide-field imaging minimizes patient and photographer burden, necessitating less photos and technical skill to capture the peripheral retina. Minimizing the number of necessary images can be especially helpful for pediatric patients with sickle cell retinopathy. Wide-field imaging has already been successful in identifying new biomarkers and risk factors for the development of proliferative sickle cell retinopathy. While these advantages should be considered, clinicians need to perform a careful risk–benefit analysis before ordering this test. Although wide-field fluorescein angiography successfully detects additional pathologic abnormalities compared to traditional imaging, a recent research study suggests that peripheral changes differentially detected by wide-field imaging may not change clinical management for most sickle cell patients. While wide-field imaging may not carry a clinically significant direct benefit to all patients, it shows future promise in expanding our knowledge of sickle cell retinopathy. Clinicians may monitor peripheral retinal pathology such as retinal ischemia and retinal neovascularization over progressive time points, and use sequential wide-field retinal images to monitor response to treatment. Future applications for wide-field imaging may include providing data to facilitate machine learning, and potential use in tele-ophthalmology screening for proliferative sickle retinopathy.

/pdf/wide-field-imaging-of-sickle-retinopathy-3tw5ci83qg.pdf

Wide-field imaging of sickle retinopathy.

Sickle cell disease (SCD) prevalence has increased rapidly in Europe as a result of an increase in the life expectancy of these patients and the arrival of SCD migrants from Africa. The aim of our study was to compare the phenotypes of adult patients born in Sub-Saharan Africa (SSA) who migrated to France with those of patients with the same origin who were born in France. This single-center observational study compared the demographic, clinical and biological characteristics of SCD adult patients of SSA origin who were born in France or SSA. Data were collected from computerized medical charts. Groups were compared using multivariate logistic regression with adjustment for age, gender and type of SCD. Of the 323 SCD patients followed in our center, 235 were enrolled, including 111 patients born in France and 124 patients born in SSA. SCD genotypes were balanced between groups. Patients born in Africa were older (median age 32.1 (24.4–39) vs. 25.6 (22.1–30.5) years, p < 0.001) and more often women (n = 75 (60.5%) vs. 48 (43.2%), p = 0.008). The median age at arrival in France was 18 years (13–23). The median height was lower among patients born in SSA (169 (163–175) vs. 174.5 cm (168–179), p < 0.001). Over their lifetimes, patients born in France had more acute chest syndromes (median number 2 (1–4) vs. 1 (0–3), p = 0.002), with the first episode occurring earlier (19 (11.6–22.3) vs. 24 (18.4–29.5) years, p < 0.007), and were admitted to intensive care units more often (53.3% vs. 34.9%, p = 0.006). This difference was more pronounced in the SS/Sβ0 population. Conversely, patients born in SSA had more skin ulcers (19.4% vs. 6.3%, p = 0.03). No significant differences were found in social and occupational insertion or other complications between the two groups. Patients born in SSA had a less severe disease phenotype regardless of their age than those born in France. This difference could be related to a survival bias occurring in Africa during childhood and migration to Europe that selected the least severe phenotypes.

Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa.

Resume Introduction La retinopathie est une complication chronique a risque fonctionnel important chez le drepanocytaire, dont l’incidence est mal connue en Afrique subsaharienne, faute de depistage. Ce travail decrit les resultats d’un depistage systematique de la retinopathie drepanocytaire dans un centre de suivi des drepanocytaires au Mali. Methodes Tous les drepanocytaires âges de 10 ans et plus, suivis au centre, ont ete depistes entre 2010 et 2012 par un examen du fond d’œil complete si besoin par une angiographie. Les caracteristiques des patients porteurs d’une retinopathie ont ete comparees a celles de drepanocytaires sans retinopathie recrutes durant la meme periode et selectionnes au hasard. Resultats La retinopathie avait une prevalence globale de 8,8 % chez les 1604 drepanocytaires âges de 10 ans et plus et concernait 91/731 (12,4 %) drepanocytaires SC, 38/734 (5,2 %) SS, 5/53 (9,4 %) Sβ 0  thalassemiques et 8/86 (9,3 %) Sβ +  thalassemiques. La forme proliferante de la retinopathie etait plus frequente chez les SC ( p 0 thalassemiques. Conclusion La retinopathie touche pres d’un patient drepanocytaire sur dix et sa prevalence est negativement associee au taux d’hemoglobine fœtale. L’efficience d’un depistage systematique de la retinopathie drepanocytaire doit etre etudiee en Afrique, ainsi que l’interet de la phlebotomie et de l’hydroxycarbamide en traitement preventif.

M. Coulibaly

Papers

Prévalence et facteurs de risque de la rétinopathie drépanocytaire dans un centre de suivi drépanocytaire d’Afrique subsaharienne