Orbital wall infarction in child with sickle cell disease

Janssens, Carole; Claeys, L.; Maes, Philip; Boiy, Tine; Wojciechowski, Marek

doi:10.1179/2295333715y.0000000053

Cited by 10 publications

(8 citation statements)

References 5 publications

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“…One other report of orbital wall infarction without orbital pain has been described, but the patient presented with pain due to SCD in other areas of the body and was already taking pain medications when periorbital swelling began [8]. Our patient presented with no pain at all.…”

Section: Discussionmentioning

confidence: 65%

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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Section: Discussionmentioning

confidence: 65%

Orbital Infarction due to Sickle Cell Disease without Orbital Pain

McBride

Kumar

2016

Case Reports in Ophthalmological Medicine

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“…The etiology of SOH is currently still misunderstood. SOH has been suggested to be related to the extravasation of blood from necrotized vessel walls, underlying bleeding diathesis, and minor trauma [ 6 , 7 , 9 , 10 ]. In the literature, SOH has been reported in several other cases of patients with SCD [ 6 , 7 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…SOH has been suggested to be related to the extravasation of blood from necrotized vessel walls, underlying bleeding diathesis, and minor trauma [ 6 , 7 , 9 , 10 ]. In the literature, SOH has been reported in several other cases of patients with SCD [ 6 , 7 , 9 ]. In these cases, orbital wall infarction with subperiosteal hemorrhage was often accompanied with eyelid swelling, with or without visual impairment.…”

Section: Discussionmentioning

confidence: 99%

Subperiosteal Orbital Hematoma: A Rare Clinical Manifestation of Sickle Cell Disease - A Case Report

Hanna,

Musleh,

Khan

et al. 2023

Case Rep Ophthalmol

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Sickle cell disease (SCD), an inherited vaso-occlusive disorder, results in recurrent painful episodes and a variety of serious systemic complications that can lead to severe disabilities and even death. Here, we report a case of a 19-year-old African American patient with homozygous sickle cell trait who presented with right upper lid edema and ptosis, 3 days after his admission to the hospital following a sickle cell crisis. Initially, mistaken as a superinfection in the context of his disease, a diagnosis of orbital abscess was made. Intravenous antibiotics and a proper treatment plan were set accordingly. Only after extensive clinical and radiological examinations, it turned out to be an acute subperiosteal orbital hematoma, a rare clinical manifestation of SCD. The aim of our case report was to highlight the difference in orbital presentation between osteomyelitis and subperiosteal hematoma, as well as spreading awareness among medical professionals and especially ophthalmologists for this rare presentation of orbital wall infarction, as the initial differential diagnosis of SCD patients with ocular involvement.

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“…Although vasoocclusive crises often affect the bone marrow of vertebrae and long bones, they may involve the orbital walls in children where the marrow content is higher. [22][23][24] Presentation may be severe pain, lid edema, proptosis, ophthalmoplegia as well as diplopia. [22][23][24][25] Orbital bone infarction can lead to an orbital hematoma, which in turn may lead to orbital compartment syndrome.…”

Section: Orbital Involvementmentioning

confidence: 99%

“…[22][23][24] Presentation may be severe pain, lid edema, proptosis, ophthalmoplegia as well as diplopia. [22][23][24][25] Orbital bone infarction can lead to an orbital hematoma, which in turn may lead to orbital compartment syndrome. 26 Both osteomyelitis and orbital cellulitis may present similarly, and thus confirmation of the diagnosis of orbital bone infarction is of great importance.…”

Section: Orbital Involvementmentioning

confidence: 99%

Hemoglobinopathies: ocular manifestations in children and adolescents

Moussa

Chen

2021

Ophthalmol Eye Dis

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Hemoglobinopathies are genetic disorders that lead to abnormal structure of the hemoglobin molecule. Sickle cell disease, the most common inherited blood disorder, is characterized by defective oxygen transport. Almost every part of the eye can be affected by sickle cell disease; however, proliferative sickle cell retinopathy is the primary cause of vision loss, either from vitreous hemorrhage or retinal detachment. Here we review the various manifestations of hemoglobinopathies on the eyes of children and adolescents, with a specific focus on sickle cell disease and its different phenotypes. Newer, more sensitive ophthalmological imaging modalities, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography, are available. These sensitive modalities allow for a more thorough examination of the retinal periphery where sickle cell retinopathy is often present. Utilization of such modalities will help with the early detection of the disease in children, which provide a better understanding of the pathogenesis of the disease and guide future screening and treatment regimens.

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Orbital wall infarction in child with sickle cell disease

Cited by 10 publications

References 5 publications

Orbital Infarction due to Sickle Cell Disease without Orbital Pain

Orbital Infarction due to Sickle Cell Disease without Orbital Pain

Subperiosteal Orbital Hematoma: A Rare Clinical Manifestation of Sickle Cell Disease - A Case Report

Hemoglobinopathies: ocular manifestations in children and adolescents

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