Makulopathie bei Sichelzellerkrankung

Bachmeier, Isabel; Blecha, Christiane; Föll, Jürgen; Wolff, Daniel; Jägle, Herbert

doi:10.1007/s00347-020-01319-8

Cited by 4 publications

(4 citation statements)

References 76 publications

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“…4 Over the last ten years, ophthalmologists involved in the care of patients with sickle cell disease (SCD) have increasingly focused their research from the periphery to the center of the retina -the macula. 5,6 While Goldbaum first reported alterations of the "macular reflex" in 1978, 7 little interest had been given to the macula of SCD patients until the development of optical coherence tomography (OCT). By providing a unique, non-invasive, in vivo visualization of the retinal layers, OCT images completed Goldbaum's initial description by showing areas of thinning, i.e.…”

Section: Introductionmentioning

confidence: 99%

Retinal atrophy and markers of systemic and cerebrovascular severity in homozygous sickle cell disease

Martin

Brousse

Connes

et al. 2022

European Journal of Ophthalmology

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Introduction While paramacular retinal atrophy (PRA) is known to be found in 48% of eyes of adults and 42% of eyes of children with homozygous SCD (SS-SCD), the aim of this study is to assess the association between PRA and red blood cell (RBC) deformability, hematological markers and brain imaging abnormalities in SS-SCD. Methods This study is a subset of DREAM2, a prospective observational study performed between August 2015 and August 2016. Children (5–17 years) with SS-SCD and no history of large vessel vasculopathy, were included. Ophthalmological characteristics including visual acuity, fundus examination, OCT of central and temporal retina (with several retinal thickness measurements) were explored in relation with RBC deformability (ektacytometry), hematological and biochemical (hemolysis parameters), and neurological (cerebral oxygenation estimated by Near Infrared Spectroscopy, brain magnetic resonance imaging) investigations. Results 17 children (5 boys; mean age: 13 years) with complete ophthalmological investigations were included in the analysis; 8 exhibited PRA. RBC deformability was found to be significantly lower in children with PRA for measurements made at 1.69 Pa (0.16 a.u ± 0.02 vs 0.21 a.u ± 0.03, p = 0.02) and above, as well as cerebral oxygenation (59.25% ± 9.9 vs 71.53% ± 4.9, p = 0.02). A significant positive correlation was found between temporal retinal thickness and hemoglobin level (ρ = 0.65, p = 0.007), hematocrit (ρ = 0.53, p = 0.04) and RBC deformability at 3 Pa (ρ = 0.75, p = 0.005) and above. Conclusions These results suggest that PRA could be an early marker of systemic severity and cerebral oxygenation in SCD. Whether it could help predicting cerebral vasculopathy requires further investigations.

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Section: Introductionmentioning

confidence: 99%

Retinal atrophy and markers of systemic and cerebrovascular severity in homozygous sickle cell disease

Martin

Brousse

Connes

et al. 2022

European Journal of Ophthalmology

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“…We did not observe any strong correlation between temporal macular thickness at 3 or 6 mm and the Goldberg stage of PSR. Bachemeier et al 27 also considered that this SCDM occurred irrespective of PSR. In addition, Mokrane et al 28 did not detect any relation between the vascular density in DCP et SCP according to the Goldberg classification.…”

Section: Discussionmentioning

confidence: 99%

Atypical Foveal and Parfoveal Abnormalities in Sickle Cell Disease

Orssaud,

Flammarion,

Michon

et al. 2023

Retina

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Purpose: The primary aim was to describe patterns of para macular involvement, not yet reported but that OCT-A can now detect in SCD patients. The secondary aim was to search arguments concerning the physio pathogeny of para macular involvement. Methods: This institutional cohort retrospective study was conducted in a Referral Center for Ophthalmological Rare Diseases. Follow-up included an ophthalmological examination with optical coherent tomography and optical coherent tomography angiography. Results: Hundred and thirty-two sickle cell patients were included. Typical sickle cell maculopathy was observed in temporal area in 84 eyes (40.0 %) of SS patients and 8 eyes (14.8 %) of SC patients (p < 0.001). Enlargement of the foveal avascular zone was observed in 10 eyes whom 8 of SS patients. Two atypical parafoveal abnormalities were found in SS patients only. The first one consisted in macular thinning with normal vascularization in 15 eyes of 11 patients. The second atypical maculopathy was large areas of loss of vascularization without retina thinning 10 eyes of six patients. Multivariate analysis did not show a statistically significant relation between the PSR stage and the different type of sickle cell maculopathy (p = 0.21) Conclusion: Those atypical sickle cell maculopathy may correspond to early forms preceding a typical SCM. This would point towards several physiopathogenic mechanisms. The first one included the existence of ischemia which can be related to anemia. Presence of retinal thinning without vascular involvement point out to a neurogenic mechanism.

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“…Other authors did not confirm such an association. Bachmeier considered that SCM occurs irrespective of PSR in a study of German patients ( 22 ). Only Lim reported that macular thinning increased with PSR stages ( 19 ).…”

Section: Discussionmentioning

confidence: 99%

Relationship between paramacular thinning, cerebral vasculopathy, and hematological risk factors in sickle cell disease

Orssaud,

Flamarion,

Michon

et al. 2023

Front. Med.

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PurposeTo identify risk factors for sickle cell maculopathy due to hematological parameters (especially anemia and hemolysis) or cerebral vasculopathy.MethodsThis retrospective study was conducted at a Referral Center. The follow-up included optical coherent tomography/optical coherent tomography angiography, neuro-radiological imaging, and a hematological assessment (hemoglobin, hemoglobin S level, reticulocytes, mean corpuscular volume, bilirubin, and lactate dehydrogenase).ResultsHundred and thirty-two sickle cell patients were included. Maculopathy was observed in 127 eyes of SS patients and 10 eyes of SC patients (p < 0.001), unrelated to peripheral retinopathy. Cerebral vasculopathy was more frequent in SS patients (p < 0.001) and was also associated with the presence of maculopathy (p = 0.049), and it was related to peripheral retinopathy (p < 0.001). All biological parameters significantly differed according to the genotype (p < 0.001) but not according to the presence of cerebral vasculopathy or maculopathy. In the multivariate analysis, reticulocytes and bilirubin were associated with the presence of cerebral vasculopathy and maculopathy.ConclusionThe data obtained were consistent with the role of anemia or hemolysis markers in cerebral vasculopathy and macular involvement. As a trend of hemolysis appears to be a risk factor for these complications, this validates the use of preventive plasmapheresis in these patients.

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Makulopathie bei Sichelzellerkrankung

Cited by 4 publications

References 76 publications

Retinal atrophy and markers of systemic and cerebrovascular severity in homozygous sickle cell disease

Retinal atrophy and markers of systemic and cerebrovascular severity in homozygous sickle cell disease

Atypical Foveal and Parfoveal Abnormalities in Sickle Cell Disease

Relationship between paramacular thinning, cerebral vasculopathy, and hematological risk factors in sickle cell disease

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