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Case 348: Orbital Subperiosteal and Epidural Hematomas Secondary to Sickle Cell Bone Infarcts
1Department of Diagnostic Radiology and Nuclear Medicine, University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201.
Insights
A child with sickle cell disease experienced eye swelling and pain, prompting imaging studies. The case highlights potential complications of sickle cell disease in pediatric patients.
Area of Science:
- Pediatric Hematology
- Ophthalmology
- Radiology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Acute chest syndrome (ACS) is a common complication of SCD.
- Orbital complications in SCD are rare but can be severe.
Purpose of the Study:
- To report a case of orbital swelling in a child with sickle cell disease.
- To discuss the diagnostic approach and potential underlying causes.
Main Methods:
- A 6-year-old boy with SCD and a history of ACS presented with right eye swelling and pain.
- Laboratory tests revealed anemia, elevated white blood cell count, and elevated liver enzymes.
- Imaging included contrast-enhanced CT of the face and MRI of the brain and orbits.
Main Results:
- Physical examination showed right eye swelling and erythema without significant pain on eye movement.
- Laboratory findings were consistent with hemolysis and inflammation.
- CT and MRI provided detailed imaging of the orbital structures.
Conclusions:
- Orbital complications should be considered in children with SCD presenting with eye swelling.
- Prompt diagnosis and management are crucial to prevent vision loss.
- Multimodal imaging plays a key role in evaluating these rare presentations.
History:
A 6-year-old boy with sickle cell disease and a recent episode of acute chest syndrome presented to the emergency department with chest tightness and a 4-day history of progressive right eye swelling, along with pain in the left hand and left leg. Initial laboratory tests showed a hemoglobin level of 6.1 g/dL (61 g/L) (low), hematocrit level of 19.5% (low), white blood cell count of 16 000/μL (16 × 109/L) (high), red blood cell count of 3.03 million/μL (3.03 × 1012/L) (low), platelet count of 338 × 103/μL (338 × 109/L) (normal), reticulocyte count of 4.79% (high), total bilirubin level of 2.3 mg/dL (39 μmol/L) (high), aspartate aminotransferase level of 62 U/L (1.0 μkat/L) (high), alanine aminotransferase level of 16 U/L (0.27 μkat/L) (normal), and alkaline phosphatase level of 660 U/L (11 μkat/L) (high). The differential count was as follows: neutrophils, 69.1% (high); lymphocytes, 22.7% (low); monocytes, 7.2% (normal); basophils, 0.3% (normal); and immature granulocytes, 0.7% (high). Physical examination demonstrated swelling and erythema of the right eye but no significant discomfort or tenderness with eye movement. There was no eye discharge or conjunctival redness. Contrast-enhanced CT of the face was performed, followed by MRI of the brain and orbits with and without intravenous contrast agent.
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