Latent proliferative sickle cell retinopathy in sickle cell trait

Mehta, J. S.; Whittaker, Karl W; Tsaloumas, M D

doi:10.1034/j.1600-0420.2001.079001081.x

Cited by 14 publications

(4 citation statements)

References 6 publications

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“…Environmental factors may confound these studies. For example, Mehta et al described a patient with SCT who developed PSR after mild ocular blunt trauma [36]. The reported prevalence of PSR in various genotypes is summarized in Table 1.…”

Section: Genetic and Systemic Risk Factors Of Sickle Cell Retinopathymentioning

confidence: 99%

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: Genetic and Systemic Risk Factors Of Sickle Cell Retinopathymentioning

confidence: 99%

Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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show abstract

“…10 Mild trauma in patients with sickle trait can also lead to latent proliferative retinopathy. 11 In our case, a review of systems and the pediatrician's evaluation failed to suggest any comorbid systemic diseases. The patient's family history was negative for familial exudative vitreoretinopathy and there was no temporal dragging of the retinal vessels.…”

Section: E60mentioning

confidence: 60%

Leukocoria and Vitreous Hemorrhage as the Initial Manifestation of Sickle Retinopathy in a 3-Year, 6-Month-Old Child With Sickle Trait (AS)

Barry¹,

Shields²,

Binenbaum³

2011

J Pediatr Ophthalmol Strabismus

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The authors present the youngest reported child with proliferative sickle retinopathy. A 3-year, 6-month-old boy presented with leukocoria in the left eye, left esotropia, and dense free-floating white vitreous cells obscuring the fundus, suspicious for endophytic retinoblastoma. Ultrasonography depicted dense debris in the vitreous with no distinct calcific echo or retinal mass. Transcorneal, transzonular fine-needle aspiration biopsy of the vitreous confirmed the presence of dehemoglobinized vitreous red blood cells without tumor. The opposite eye showed peripheral retinal pigment epithelial proliferation and fibrosis with angiographic peripheral ischemia. Hemoglobin electrophoresis disclosed sickle trait (AS). The findings were classified as proliferative sickle cell retinopathy with vitreous hemorrhage in the left eye and non-proliferative sickle cell retinopathy in the right eye. Leukocoria generally raises warnings for retinoblastoma, but can also reflect chronic vitreous hemorrhage.

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“…The frequency of retinopathy is greatest in adulthood, but retinopathy has also been described in children [6]. Individuals with sickle-cell trait comprise 8% of the African-Caribbean [7]. Although these individuals often show no relevant clinical manifestations, they may present various retinal changes such as hemorrhages, exudates, angioid streaks, acute chorioretinal infarction, chorioretinitis, and vitreous hemorrhage, retinal vascular abnormalities such as tortuous / dilated retinal veins, microaneurysms, central retinal artery occlusion and retinal proliferation [7].…”

Section: Methodology Epidemiology and Pathogenesismentioning

confidence: 99%

Retinal occlusion as an advanced complication of sickle cell disease

Alkhaibari¹

2016

New Front Ophthalmol

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Retinopathy is a one of the major clinical manifestation of Hemoglobinopathy. It is acquired secondary to another retinal disorder. Retinopathy especially retinal occlusions are painless loss of monocular vision it's from a vascular disorder. Ocular stroke caused by embolism in a retinal artery, that may emboli travel to distal branches of the retinal artery, causing loss of other section in the visual field. The manifestations of Sickle cell disease ocular manifestations came due to vascular occlusion, which may exist in the conjunctiva, iris, retina, and choroid. Because the ocular changes produced by SCD could be shown in other diseases, it's important to except other occlusions' causes, which have included central retinal vein occlusion, Eales disease, and retinopathy secondary to other chronic disorders. Other ocular changes cause that includes polycythemia vera, familial exudative vitreoretinopathy, talc and cornstarch emboli, and uveitis. Diagnosis of hemoglobenopathies is performed exclusively through Hb electrophoresis. The treatments and their results vary from one condition to the other.

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Latent proliferative sickle cell retinopathy in sickle cell trait

Cited by 14 publications

References 6 publications

Sickle cell retinopathy. A focused review

Sickle cell retinopathy. A focused review

Leukocoria and Vitreous Hemorrhage as the Initial Manifestation of Sickle Retinopathy in a 3-Year, 6-Month-Old Child With Sickle Trait (AS)

Retinal occlusion as an advanced complication of sickle cell disease

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