Paramacular temporal atrophy in sickle cell disease occurs early in childhood

Martin, Gilles C.; Albuisson, Éliane; Brousse, Valentine; Montalembert, Mariane de; Bremond-Gignac, Dominique; Robert, Matthieu P.

doi:10.1136/bjophthalmol-2018-312305

Cited by 14 publications

(21 citation statements)

References 22 publications

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“…Spectral-domain optical coherence tomography and optical coherence tomography angiography (SD-OCT and OCT-A) identified abnormal macular thinning and flow voids in the superficial and deep retinal capillary plexus in 20% of eyes that had been unobserved by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected [49,52]. Temporal macular thinning caused by paracentral acute middle maculopathy may be the result of occlusions at the level of the deep retinal capillary plexus (Fig.…”

Section: Subclinical Retinopathy and Recent Imaging Modalitiesmentioning

confidence: 77%

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: Subclinical Retinopathy and Recent Imaging Modalitiesmentioning

confidence: 77%

Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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“…100 In a retrospective series of 81 children (mean age 12 years; standard deviation [SD]: 3.5 years) with SCD (64 HbSS, 7 HbS/β 0 -thalassemia, 10 HbSC), temporal macular retinal atrophy was observed in 64% of cases with no significant difference in prevalence between HbSS and HbSC genotype. 101 In this series, peripheral retinopathy was observed in 11% of patients and was more severe in children with HbSC.…”

Section: Ophthalmic Diseasementioning

confidence: 54%

Chronic organ injuries in children with sickle cell disease

et al. 2021

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Median life expectancy of patients with sickle cell disease has increased to up to 55 years but there are still frequent cases of premature death, mostly in patients with pre-existing organ failure such as pulmonary hypertension, kidney injury, and cerebral vasculopathy. Most organ injuries remain asymptomatic for a long time and can only be detected through early systematic screening. Protocols combining assessment of velocities on transcranial Doppler and regular transfusions in patients with abnormal velocities have been demonstrated to dramatically reduce the risk of stroke. In contrast, no consensus has been reached on systematic screening or therapy for silent cerebral infarcts. The prognostic significance of increased tricuspid regurgitant jet velocity on echocardiography has not yet been identified in children, whereas increased albuminuria is a good predictor of kidney injury. Finally, screening for hip and eye disorder is recommended; however, different countries adopt different screening strategies. Hydroxyurea is probably of potential benefit in preventing chronic organ damage but this requires further study in order to be fully demonstrated. Efficacy and safety of the other new drugs available are also under investigation.

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Section: Sickle Cell Maculopathy • Macular Thinning • Retinopathy • Ounclassified

“…Aus einer Studie von Martin et al geht hervor, dass die SZM bereits früh in der Kindheit auftritt [45] und damit deutlich früher als die periphere SZR, für die der Gipfel des Auftretens im Jugendoder frühen Erwachsenenalter liegt [7]. Die Prävalenz der SZM wird mit bis zu 64 % der SZE-Patienten angegeben [45]. Sie betrifft Patienten mit HbSS-SZE möglicherweise häufiger als Patienten mit HbSC-SZE [46], während es sich bei der PSR genau andersherum verhält [42].…”

Section: Sickle Cell Maculopathy • Macular Thinning • Retinopathy • Ounclassified

“…Auch wenn eine temporale Makulaverdünnung unabhängig vom gleichzeitigen Vorliegen einer PSR auftreten kann, so scheint sie dennoch mit dem Grad peripherer Nonperfusion zu korrelieren und mit einem höheren Risiko einer PSR vergesellschaftet zu sein [46]. Auch wurde das Vorliegen einer SZM mit einem vermehrten Auftreten von ze-rebrovaskulären Komplikationen assoziiert, wodurch die SZM trotz asymptomatischen Verlaufs und trotz fehlender Therapieoptionen klinische Relevanz erlangt [45].…”

Section: Sickle Cell Maculopathy • Macular Thinning • Retinopathy • Ounclassified

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Makulopathie bei Sichelzellerkrankung

et al. 2021

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Zusammenfassung Hintergrund Die Sichelzellerkrankung (SZE) ist eine hereditäre Hämoglobinopathie, die durch rezidivierende vasookklusive Episoden zur Mikrozirkulationsstörung verschiedener Organsysteme mit teils letalem Ausgang führt. Bei der okulären Manifestation der SZE ist am bekanntesten die periphere Sichelzellretinopathie (SZR). Unabhängig davon kann es bereits früh im Krankheitsverlauf zur Sichelzellmakulopathie (SZM) kommen. Methoden Review der internationalen und deutschsprachigen Literatur zur okulären Beteiligung bei SZE mit Fokus auf die SZR und SZM sowie Überblick über aktuelle systemische Therapieansätze bei SZE anlässlich der Vorstellung zweier Patienten mit HbSS-SZE. Ergebnis und Schlussfolgerung Im Gegensatz zur SZR ist die SZM mit temporaler Verdünnung der inneren Netzhautschichten erst in den letzten 5 Jahren mit der Einführung von SD-OCT und OCTA vermehrt in die Literatur eingegangen. Unabhängig vom Vorliegen einer SZR kann es immerhin bei etwa der Hälfte der Patienten bereits früh im Krankheitsverlauf zu einer SZM kommen. Das Krankheitsbild wird auch in Deutschland durch den Fortschritt der systemischen Therapiemöglichkeiten und aufgrund von Migration präsenter werden. Durch Wissen um diese Komplikation der SZE kann eine frühzeitige Diagnosestellung erfolgen und unnötige Diagnostik vermieden werden.

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Paramacular temporal atrophy in sickle cell disease occurs early in childhood

Abstract: Paramacular temporal atrophy occurs early in the course of SCD, which suggests distinct mechanisms from those of peripheral retinopathy.

Cited by 14 publications

References 22 publications

Sickle cell retinopathy. A focused review

Sickle cell retinopathy. A focused review

Chronic organ injuries in children with sickle cell disease

Makulopathie bei Sichelzellerkrankung

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