Case Report: Expanding the ocular horizon: subperiosteal orbital hematoma and Roth spots in known case of sickle cell disease

Sickle cell disease is a prevalent haemoglobinopathy that is hereditary. People with sickle cell disease frequently experience vaso-occlusive crises, which lead to substantial morbidity from end-organ ischaemia and infarction. Numerous organs, including the bones, brain, lungs, spleen, retina, and penis, may be affected. As a result, symptoms may include painful bone crises, cerebrovascular accidents, acute chest syndrome, sequestration crises, retinal haemorrhage, and priapism. In sickle cell disease, subperiosteal orbital haemorrhage is a rare vaso-occlusive crisis (VOC). Roth spots (white-centred, flame-shaped retinal haemorrhages), occurring commonly in subacute bacterial endocarditis, can also be seen in leukaemia, pre-eclampsia, and hypertensive retinopathy. A 19-year-old male patient with sickle cell disease (HbSS) presented to the hospital with ptosis and swelling in his right periorbital region 3 days after being brought to the hospital for a sickle cell crisis. It was later determined to be an acute subperiosteal orbital hematoma along with Roth spots, an uncommon clinical manifestation of sickle cell disease, only after a thorough clinical and radiological assessment.

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Journal
Frontiers in Hematology
Published
2025-06-16
DOI
https://doi.org/10.3389/frhem.2025.1558509
Primary Topic
Hemoglobinopathies and Related Disorders
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article
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article

Case Report: Expanding the ocular horizon: subperiosteal orbital hematoma and Roth spots in known case of sickle cell disease

Rajvardhan Patil, Sourya Acharya, Vineet Karwa, Rushikesh H Dhondge et al.
Frontiers in Hematology
Hemoglobinopathies and Related Disorders
article

Case Report: Expanding the ocular horizon: subperiosteal orbital hematoma and Roth spots in known case of sickle cell disease

Rajvardhan Patil, Sourya Acharya, Vineet Karwa, Rushikesh H Dhondge, Suhail Shaikh, M. Kothari
article en

Abstract

Sickle cell disease is a prevalent haemoglobinopathy that is hereditary. People with sickle cell disease frequently experience vaso-occlusive crises, which lead to substantial morbidity from end-organ ischaemia and infarction. Numerous organs, including the bones, brain, lungs, spleen, retina, and penis, may be affected. As a result, symptoms may include painful bone crises, cerebrovascular accidents, acute chest syndrome, sequestration crises, retinal haemorrhage, and priapism. In sickle cell disease, subperiosteal orbital haemorrhage is a rare vaso-occlusive crisis (VOC). Roth spots (white-centred, flame-shaped retinal haemorrhages), occurring commonly in subacute bacterial endocarditis, can also be seen in leukaemia, pre-eclampsia, and hypertensive retinopathy. A 19-year-old male patient with sickle cell disease (HbSS) presented to the hospital with ptosis and swelling in his right periorbital region 3 days after being brought to the hospital for a sickle cell crisis. It was later determined to be an acute subperiosteal orbital hematoma along with Roth spots, an uncommon clinical manifestation of sickle cell disease, only after a thorough clinical and radiological assessment.

Frontiers in HematologyVol. 4
Jawaharlal Nehru Medical College (IN)
Openalex Percentile: Top 80%
Hemoglobinopathies and Related Disorders
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