Risk Factors for Visual Impairment in Patients with Sickle Cell Disease in London

Saidkasimova, Shohista; Shalchi, Zaid; Mahroo, Omar A.; Shunmugam, Manoharan; Laidlaw, D A H; Williamson, Tom H.; Howard, John; Mohamed, Moin

doi:10.5301/ejo.5000767

Cited by 15 publications

(22 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although HbSC patients have greater risk of proliferative retinopathy, in a recent study, HbSC genotype was not found to be a risk factor for visual loss. This may be explained in part by HbSS patients having more macular pathology contributing to sight loss, which would be supported by the findings of the present study 10 . Using OCT angiography, Han et al found lower density of deep capillary plexus at the temporal macula in HBSC eyes but no statistical difference in mean foveal nonflow area between HBSS and HBSC eyes 11 …”

Section: Discussionsupporting

confidence: 79%

Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype

Lim

Magan

Mahroo

et al. 2018

Canadian Journal of Ophthalmology

Self Cite

View full text Add to dashboard Cite

ObjectiveTemporal macula thinning has been reported in sickle cell patients, but it remains unclear if there is a difference between HbSS and HbSC genotypes. We aimed to quantitatively compare macular thickness between eyes with HbSS and HbSC genotype.DesignRetrospective descriptive study.MethodsConsecutive patients seen over a 5.5-year period in the Ophthalmology Department at St Thomas’ Hospital, London, were identified. Macular optical coherence tomography images were retrospectively analyzed. The retinal thickness in all 9 subfields of the Early Treatment Diabetic Retinopathy Study (ETDRS) grid was compared between HbSS and HbSC eyes. Right eyes and left eyes were analyzed independently, as well as averaged measurements from both eyes. Comparison was made between the 2 genotypes, adjusting for age and sex, and for multiple testing. Scans were excluded in cases of poor fixation or ocular comorbidity affecting retinal thickness.Results132 HbSC and 120 HbSS patients were identified. Scans from 166 right and 153 left eyes were included (with approximately equal numbers of HbSS and HbSC genotypes). Mean retinal thickness was lower in HbSS eyes compared with HbSC eyes in all subfields of the ETDRS grid, but in most subfields the difference was <10 microns. Differences reached statistical significance for outer superior, inferior, and temporal subfields and the inner temporal subfield (p < 0.05).ConclusionAlthough the HbSC genotype is more strongly associated with proliferative retinopathy, HbSS patients had on average more macular thinning.

show abstract

Section: Discussionsupporting

confidence: 79%

Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype

Lim

Magan

Mahroo

et al. 2018

Canadian Journal of Ophthalmology

Self Cite

View full text Add to dashboard Cite

show abstract

“…PSR was associated with older age (> 35 years) in a London cohort of 189 patients with SCD [32]. Visual impairment was related to the presence of PSR; however, it was not related to Hb genotype [32]. The association between age and progression towards PSR was further corroborated in a recent retrospective study of 296 patients with SCD, where male gender and older age were associated with PSR [33].…”

Section: Genetic and Systemic Risk Factors Of Sickle Cell Retinopathymentioning

confidence: 72%

Section: Genetic and Systemic Risk Factors Of Sickle Cell Retinopathymentioning

confidence: 90%

Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

View full text Add to dashboard Cite

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.

show abstract

“…In a recent study the most important risk factors for retinopathy were found to be HbSC genotype and age > 35 years comparable with the observations in our cohort. 26 Elevated TRV increased from 33.3% at baseline in 2006 to 37% in all patients at the final assessment after seven years of follow up. Given the median age of our cohort at the final analysis this is comparable to what has described in several cross-sectional previous study cohorts ranging from 27% to 33%.…”

Section: Ajh Ajh | E585mentioning

confidence: 88%

Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven‐year follow‐up study

et al. 2017

View full text Add to dashboard Cite

Organ damage in sickle cell disease (SCD) is a crucial determinant for disease severity and prognosis. In a previous study, we analyzed the prevalence of SCD-related organ damage and complications in adult sickle cell patients. We now describe a seven-year follow-up of this cohort.All patients from the primary analysis in 2006 (n = 104), were included for follow-up. Patients were screened for SCD-related organ damage and complications (microalbuminuria, renal failure, elevated tricuspid regurgitation flow velocity (TRV) (≥2.5 m/seconds), retinopathy, iron overload, cholelithiasis, avascular osteonecrosis, leg ulcers, acute chest syndrome (ACS), stroke, priapism and admissions for vaso-occlusive crises (VOC) biannually. Upon 7 years of follow-up, progression in the prevalence of avascular osteonecrosis (from 12.5% to 20.4%), renal failure (from 6.7% to 23.4%), retinopathy (from 39.7% to 53.8%) was observed in the whole group. In HbSS/HbSβ -thal patients also progression in microalbuminuria (from 34% to 45%) and elevated TRV (from 40% to 48%) was observed while hardly any progression in the prevalence of cholelithiasis, priapism, stroke or chronic ulcers was seen. The proportion of patients with at least one episode of ACS increased in the group of HbSS/HbSβ -thal patients from 32% to 49.1%. In conclusion, 62% of the sickle cell patients in this prospective cohort study developed a new SCD-related complication in a comprehensive care setting within 7 years of follow-up. Although the hospital admission rate for VOC remained stable, multiple forms of organ damage increased substantially. These observations underline the need for continued screening for organ damage in all adult patients with SCD.

show abstract

Risk Factors for Visual Impairment in Patients with Sickle Cell Disease in London

Abstract: We present the largest study of ocular findings in SCD in the United Kingdom. Sight-threatening PSR is a risk factor for visual impairment, but hemoglobin genotype status is not.

Cited by 15 publications

References 25 publications

Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype

Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype

Sickle cell retinopathy. A focused review

Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven‐year follow‐up study

Contact Info

Product

Resources

About