Cerebral fat embolism syndrome (CFES) is a rare but severe complication, most commonly associated with long bone fractures. Its occurrence in patients with sickle cell disease (SCD) is exceptional and may be triggered by vaso-occlusive crises. Its clinical manifestations are variable and nonspecific, frequently overlapping with stroke, sepsis, and other SCD-related complications, making diagnosis particularly challenging. We report the case of a 56-year-old woman with SCD admitted for vaso-occlusive crisis who developed rapid neurological deterioration with encephalopathy and tetraplegia. Brain MRI revealed both small punctate DWI hyperintensities and innumerable petechial hemorrhages on SWI, highly suggestive of CFES. Additional imaging demonstrated vertebral osteonecrosis, bone marrow infarction, and extensive iliac and femoral venous thrombosis, pointing to widespread vaso-occlusion as the embolic source. The patient was treated with exchange transfusion, anticoagulation, targeted antibiotic therapy, and rehabilitation, resulting in gradual clinical stabilization. This case highlights the diagnostic value of susceptibility imaging, as SWI-detected microhemorrhages predominated over the classic “starfield” pattern on DWI and may serve as a more consistent marker across disease stages. To our knowledge, this report represents one of the few cases of CFES in patients with SCD in which SWI findings clearly predominated over DWI abnormalities and were documented longitudinally. A normal brain CT does not exclude CFES, and in patients with SCD presenting with acute neurological deterioration, early MRI, particularly including SWI, is essential. Raising awareness of this imaging profile is crucial for the timely recognition and effective management of CFES in SCD, with potential implications for patient outcomes.
Failla et al. (Wed,) studied this question.