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Epidural hematoma (EDH) classically occurs secondary to trauma. Spontaneous EDH is uncommon and can
be a rare complication of sickle cell disease (SCD). We report the case of a 20-year-old Indian male with
sickle cell anemia, who presented with a sickling bony crisis and suffered a non-traumatic EDH within 24
hours of admission.
A 20-year-old male presented with generalized body pain, suggestive of a sickling bony crisis. He was

promptly admitted and received standard treatment for the same. The next day, he developed severe right-
sided headache, associated with orbital pain, decreased movements on the right side, and altered sensorium.

He had a Glasgow coma scale score of 8/15, and reduced power of the right upper limb and lower limb.
Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed a left-sided large
parieto-temporal epidural hematoma with midline shift and mass effect. He underwent emergency
decompressive craniotomy and evacuation of the hematoma, following which he recovered well, with no
residual deficits.
Spontaneous EDH is being increasingly reported in SCD. Possible mechanisms include skull bone infarction,
altered skull bone anatomy due to extramedullary hematopoiesis, and venous congestion due to sluggish
blood flow in diploic veins. In our patient, altered skull anatomy appeared to be the causative mechanism.
Early identification of EDH and aggressive neurosurgical management is crucial to survival and a good
prognosis.