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Case 348
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, indicating a potential complication. Prompt imaging confirmed orbital involvement, highlighting the need for vigilant monitoring in these patients.
Area of Science:
- Pediatric Hematology
- Ophthalmology
- Radiology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with various complications.
- Acute chest syndrome is a common SCD complication, but orbital involvement is less frequently reported.
- Early recognition of SCD complications is crucial for timely intervention.
Purpose of the Study:
- To report a case of orbital complication in a child with sickle cell disease.
- To highlight the diagnostic utility of advanced imaging in evaluating such cases.
- To emphasize the importance of considering SCD complications in pediatric patients presenting with relevant symptoms.
Main Methods:
- Case report of a 6-year-old boy with SCD and acute chest syndrome.
- Clinical presentation included right eye swelling, pain, and elevated inflammatory markers.
- Diagnostic imaging included contrast-enhanced CT of the face and MRI of the brain and orbits.
Main Results:
- Physical examination revealed right eye swelling and erythema.
- Laboratory tests showed anemia, elevated white blood cell count, and elevated liver enzymes.
- Imaging confirmed orbital involvement, consistent with a complication of sickle cell disease.
Conclusions:
- Orbital complications can occur in children with sickle cell disease, even after acute chest syndrome.
- Advanced imaging like CT and MRI are essential for diagnosing orbital involvement in SCD.
- Prompt diagnosis and management are critical to prevent long-term sequelae.
Abstract:
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 (Fig 1), followed by MRI of the brain (Fig 2) and orbits (Fig 3) with and without intravenous contrast agent.
