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

Abstract

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.

Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity

“…In adults with sickle cell disease, Dell'Arti et al [9] reported no association between SCM and age, gender, or genotype; in contrast, Mathew et al [8] reported positive associations with both younger age and HbSS genotype. Others have also reported an association with the HbSS genotype [17,18]. In our study population, there was no clear association between SCM and PSR (Goldberg stages 0, 1, and 2 vs. 3, 4, and 5).…”

“…Although thinning of the inner nuclear layer is the most obvious feature of SCM on qualitative review of the structural OCT images, Dell’Arti et al [9]reported significant thinning of multiple retinal layers from the ganglion cell layer to the outer nuclear layer in eyes with SCM compared to eyes without SCM. Several authors have described thinning of a range of ETDRS subfields in the eyes of cases with sickle cell disease, especially in those with the HbSS genotype, although the absolute differences appear small [18-20]. This study identified non-significant thinning of the central 1-mm subfield in the eyes with SCM, compared to those without.…”

Central macular thickness in patients with sickle cell disease and no signs of retinopathy: a cross-sectional study of Jordanian patients

“…To the best of our knowledge, this is the first investigation of central retinal thickness measurements in Jordanian patients with sickle cell disease. Although patients with sickle cell disease may exhibit asymptomatic proliferative retinopathy, 7 we included patients without clinically evident retinopathy in this study to investigate potential tomographic changes during early stages of disease in those eyes. Future studies should examine the progression of those changes over time, their relationships with the stages of overall retinopathy, and the effects of disease-modifying agents and/or bone marrow transplantation.…”

Evaluation of retinal nerve fiber layer and choroidal thickness with spectral domain optical coherence tomography in children with sickle cell anemia

“…A total of 75 children, 30 SCA patients (Group 1), and 45 healthy individuals (Group 2), were included in the study. The mean age was 14.11±3.86 (11)(12)(13)(14)(15)(16)(17)(18) in sickle cell anemia patients and 13.15±2.69 (10)(11)(12)(13)(14)(15)(16)(17)(18) in the healthy control group. Of the patients, 56.6% (n=17) of Group 1 and 44.4% (n=20) of Group 2 were male.…”

“…11 While homozygous hemoglobin S disease (Hb SS) is associated with more severe clinical symptoms in patients with SCA, Hb SC disease has been reported to be associated with more severe and earlier retinal disease. 2,17 Lim et al 18 stated that patients with the Hb SC genotype were more prone to developing proliferative retinopathy, but macular thinning was observed more in Hb SS patients. More vaso-occlusive events were observed occuring in Hb SS patients; however, it was noted that proliferative retinopathy was paradoxically less developed in these patients.…”