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A middle childhood boy with homozygous sickle cell disease (SCD) on hydroxyurea, previously healthy, presented with acute bilateral limb pain progressing rapidly to altered sensorium and signs of raised intracranial pressure. Cranial CT revealed bilateral epidural haematomas with a large left fronto-parietal epidural haematoma (EDH) and midline shift. He underwent urgent craniotomy, mechanical ventilation, blood transfusion and phenobarbitone coma for autonomic instability. He recovered with initial right hemiparesis that improved to independent ambulation without limitation, with no recurrence on follow-up. This case represents the youngest documented survivor of spontaneous EDH in SCD, emphasising that non-traumatic intracranial bleeding can occur even at early ages in SCD patients. Emergency physicians should remain alert to this rare possibility in young children with SCD.