Spontaneous epidural hematoma (EDH) is a rare sickle cell disease (SCD) complication. We report 3 pediatric cases with SCD and spontaneous EDH and 1 with subgaleal hematomas in the setting of vaso-occlusive crises and elaborate on their presentation and management. Through a scoping review, we identified 71 additional cases reported from 1970 to 2024 and highlighted notable features. We highlight skull infarction as a prognostic factor associated with 88% lower odds of death relative to patients without skull infarction (P<0.01). We clarify optimal management strategies like early and aggressive treatment of disseminated intravascular coagulation (DIC), as 57% of patients with DIC died such that DIC was associated with 7.56 times higher odds of death (P=0.01).
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