Fatal spontaneous bilateral extradural hematoma with swirl sign in a child with sickle cell disease: lessons from delayed surgical access at a tertiary center.
Grace Ella Armande Djonde, Landry Drogba, Wilfried Meuga et al.
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Case in brief
A 13-year-old girl with homozygous sickle cell disease presented with sudden altered consciousness and left hemiparesis, showing a Glasgow coma scale of 9. Non-contrast CT revealed bilateral acute extradural hematomas, with a significant right-sided component exhibiting the swirl sign. Surgical evacuation was delayed over 12 hours due to payment issues, leading to further neurological decline. Despite successful evacuation, the patient experienced acute desaturation and hemodynamic instability, resulting in death before extubation.
What made this case unusual
This case is notable for the first description of the swirl sign in spontaneous extradural hematoma associated with sickle cell disease, indicating active bleeding.
Diagnostic / clinical pearl
Clinicians should be aware that the swirl sign on CT may indicate active bleeding in spontaneous extradural hematomas, even in the context of sickle cell disease.
Why it matters
The case underscores the critical impact of timely access to emergency neurosurgical care, particularly in resource-limited settings, on patient outcomes.