Skull infarction and epidural hematomas in a patient with sickle cell anemia

Purpose: Epidural hematomas are unusual manifestations of sickling disorders. We report a patient with sickle cell anemia and multiple skull infarctions associated with epidural hematomas. The association of skull infarctions and epidural hematomas in sickling hemoglobinopathies is reviewed. Patients and Methods: A 14-year-old boy with hemoglobin SS presented with lower back pain, left hip pain, headache, and fever. A bone scan was used to evaluate the patient for possible osteomyelitis. Head computed tomography (CT) and magnetic resonance imaging (MRI) were employed to delineate intracranial pathology. Results: The bone scan showed multiple areas of decreased uptake in the skull consistent with acute infarction before abnormalities were present on physical examination. CT scan showed a bony contour deformity of the right frontal bone, suggestive of infarction. A right frontal extra-axial collection of blood was also observed below the bony abnormality. MRI further delineated bilateral frontal and left parietal collections adjacent to the bony abnormalities consistent with subacute epidural hematomas. Conclusions: This case emphasizes the need to recognize skull infarctions and epidural hematomas as rare but potential complications of sickle cell disease. The diagnosis was facilitated by MRI, which has not been used in previous cases of skull infarctions. Moreover, our patient was successfully managed without surgical intervention.