Fatal spontaneous bilateral extradural hematoma with swirl sign in a child with sickle cell disease: lessons from delayed surgical access at a tertiary center.

BACKGROUND

Spontaneous extradural hematoma (EDH) is a rare neurosurgical complication of sickle cell disease (SCD), with bilateral pediatric presentations rarely reported. The "swirl sign" on non-contrast CT, a marker of active bleeding and independent predictor of poor prognosis in traumatic EDH and in spontaneous intracerebral hemorrhage, has never been described in spontaneous SCD-related EDH. In low-resource settings, equitable and timely access to emergency neurosurgery remains a critical and modifiable determinant of outcome.

CASE DESCRIPTION: A 13-year-old girl with homozygous SCD (HbSS) presented with sudden altered consciousness (Glasgow coma scale 9) and left hemiparesis, without history of trauma. Hemoglobin was 8 g/dL with normal hemostasis. Non-contrast CT revealed bilateral acute EDHs, with a large compressive right-sided component exhibiting the swirl sign. Emergency surgical evacuation was indicated, but delayed by over 12 h due to upfront, out-of-pocket payment requirements. During this delay, GCS dropped from 9 to 7 with right-sided mydriasis. Right fronto-parietal craniotomy with hematoma evacuation was performed. Upon scalp and pericranial reflection, the external calvarial cortex displayed an abnormal grayish-blue mottled discoloration. The bone was strikingly friable on manipulation. The hematoma was completely evacuated. At the end of the procedure, during skin closure and still under general anesthesia, the patient developed acute desaturation and hemodynamic instability and died before extubation. Autopsy was not performed.

CONCLUSIONS

This is, to our knowledge, the first description of the swirl sign in spontaneous SCD-related EDH, suggesting that this radiological marker may also indicate active bleeding in this rare clinical entity. The unusual macroscopic appearance and friability of the calvarial bone raise the possibility of a bone marrow-related perioperative complication, including fat embolism, although the cause of death could not be established. The documented neurological deterioration during the delay to surgery highlights the potential impact of delayed access to emergency neurosurgical care in resource-limited settings such as ours.