Bilateral orbital infarction and retinal detachment in a previously undiagnosed sickle cell hemoglobinopathy African child

Helen, Onakpoya Oluwatoyin; Ajite, Adebukola Bidemi; Oyelami, OA; Asaleye, CM; Adeoye, AO

doi:10.4103/0300-1652.114571

Cited by 10 publications

(8 citation statements)

References 10 publications

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“…Exudative retinal detachment can be present in SCD-induced orbital bone infarction as a result of orbito-ocular inflammation. There may be optic nerve compression [22]. The differential diagnosis includes periorbital or orbital cellulitis, orbital abscess, or allergic reaction.…”

Section: Orbital Manifestations Of Scdmentioning

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: Orbital Manifestations Of Scdmentioning

confidence: 99%

Sickle cell retinopathy. A focused review

Elsayed

Mura

Dhibi

et al. 2019

Graefes Arch Clin Exp Ophthalmol

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“… 4 A few cases of OCS have been reported in sub‐Sahara African countries, including Sudan 5 and a similar presentation in Nigeria. 6 To our knowledge, this is the first case of OCS from Uganda in the literature despite the high burden of SCD in the country.…”

Section: Introductionmentioning

confidence: 72%

Orbital compression syndrome in a Ugandan child with sickle cell disease: A case report

Olum

Nabaggala

Mwebe

et al. 2021

Clinical Case Reports

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“…Further confusing the diagnosis, sickle cell orbital infarction is closely mimicked by a variety of pathologies including osteomyelitis [7] and orbital cellulitis [5]. The unique painless nature of this case and physical and CT findings rendered us unable to rule out orbital cellulitis; subsequently, empiric antibiotics were given.…”

Section: Discussionmentioning

confidence: 99%

“…Soft tissue swelling of the orbit can result in proptosis and attenuation of extraocular movements [3–6]. Expedient diagnosis of sickle cell orbital infarction is crucial because this is a potentially sight-threatening entity [5]. …”

Section: Introductionmentioning

confidence: 99%

Orbital Infarction due to Sickle Cell Disease without Orbital Pain

McBride

Kumar

2016

Case Reports in Ophthalmological Medicine

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Sickle cell disease is a hemoglobinopathy that results in paroxysmal arteriolar occlusion and tissue infarction that can manifest in a plurality of tissues. Rarely, these infarcted crises manifest in the bony orbit. Orbital infarction usually presents with acute onset of periorbital tenderness, swelling, erythema, and pain. Soft tissue swelling can result in proptosis and attenuation of extraocular movements. Expedient diagnosis of sickle cell orbital infarction is crucial because this is a potentially sight-threatening entity. Diagnosis can be delayed since the presentation has physical and radiographic findings mimicking various infectious and traumatic processes. We describe a patient who presented with sickle cell orbital crisis without pain. This case highlights the importance of maintaining a high index of suspicion in patients with known sickle cell disease or of African descent born outside the United States in a region where screening for hemoglobinopathy is not routine, even when the presentation is not classic.

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Bilateral orbital infarction and retinal detachment in a previously undiagnosed sickle cell hemoglobinopathy African child

Cited by 10 publications

References 10 publications

Sickle cell retinopathy. A focused review

Sickle cell retinopathy. A focused review

Orbital compression syndrome in a Ugandan child with sickle cell disease: A case report

Orbital Infarction due to Sickle Cell Disease without Orbital Pain

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