Spontaneous epidural hematoma secondary to bone infarction in sickle cell anemia: case report

Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation. A second companion case is also presented. The authors discuss proposed pathophysiology of the formation of EDHs in sickle cell anemia.