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Breaking age barriers: spontaneous epidural haematoma in a child with sickle cell disease
Bipin Bihari Rout1, Santosh Navi1, Raju S M1
1Paediatrics, All India Institute of Medical Sciences Raipur, Raipur, Chhattisgarh, India.
Insights
A young boy with sickle cell disease (SCD) survived a spontaneous, non-traumatic epidural hematoma (EDH), highlighting the need for vigilance in pediatric SCD patients. This case represents the youngest documented survivor of such a critical event.
Area of Science:
- Pediatric Neurology
- Hematology
- Neurosurgery
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with various complications.
- Intracranial bleeding is a known but rare complication of SCD, particularly in younger patients.
- Spontaneous epidural hematomas (EDH) are uncommon in children, especially those with SCD.
Purpose of the Study:
- To report the case of the youngest documented survivor of spontaneous EDH in a child with SCD.
- To emphasize the possibility of non-traumatic intracranial bleeding in pediatric SCD patients.
- To raise awareness among emergency physicians regarding this rare but critical condition.
Main Methods:
- Case report of a middle childhood boy with homozygous SCD.
- Clinical presentation including limb pain, altered sensorium, and signs of increased intracranial pressure.
- Diagnostic imaging (Cranial CT) revealing bilateral EDH with midline shift.
- Emergency surgical intervention (craniotomy) and medical management (mechanical ventilation, blood transfusion, phenobarbitone coma).
Main Results:
- The patient survived a large left fronto-parietal EDH with midline shift.
- Successful surgical evacuation of the hematoma and intensive medical management.
- Initial recovery with right hemiparesis, which significantly improved to independent ambulation.
- No recurrence of EDH on follow-up.
Conclusions:
- This case represents the youngest documented survivor of spontaneous EDH in SCD.
- Non-traumatic intracranial bleeding, including EDH, can occur in young SCD patients.
- Emergency physicians must consider EDH in the differential diagnosis of pediatric SCD patients presenting with neurological deterioration.
Abstract:
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.
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