RESUMO
OBJECTIVE: To report a rare case of spontaneous bilateral epidural hematoma (EDH) in a 10-year-old Nigerian child with sickle cell disease (SCD) and review the literature regarding this unusual complication. METHODS: We present a case of a pediatric patient with SCD who developed a spontaneous bilateral EDH and discuss the potential underlying mechanisms, management approaches, and outcomes. We also conducted a literature review of existing cases of spontaneous EDH in patients with SCD. RESULTS: Our patient initially presented with a subgaleal hematoma and underlying bilateral EDH, but she was sent home without any neurosurgical consultation. Two years later, she returned with altered consciousness and left-sided weakness, revealing an increased size of the EDH with a noticeable mass effect. She underwent a successful emergency bilateral craniotomy, with noticeable improvement in her level of consciousness and left-sided weakness post-operatively. In our literature review, we found 40 reported cases of spontaneous EDH in SCD patients, with a male predominance (82.5%). The average age of patients was 15.282 years. The most common hematoma location was bifrontal (20%) and the most reported symptom was headache (47.5%). Most patients (97.5%) were already known cases of SCD. Among those treated, 77.5% survived. CONCLUSION: Spontaneous bilateral EDH in SCD patients is an uncommon complication, with a variety of proposed pathophysiological mechanisms. Prompt recognition and appropriate management, either conservative or surgical, are crucial to improve outcomes. Our case and literature review underscore the importance of considering spontaneous EDH in SCD patients presenting with neurological symptoms, even in the absence of trauma. Further research is needed to elucidate the precise etiology, identify risk factors, and optimize management approaches for this rare complication in SCD patients.
