Objective:

To report a patient with Sickle Cell Disease who developed Cerebral Fat Embolism Syndrome.

Background:

A review of literature showed that cerebral fat embolism syndrome (CFES) can occur as a symptom of bone necrosis and vaso-occlusive crisis in patients with sickle cell disease (SCD), especially in those with heterozygous variants.

Design/Methods:

Case report and literature review

Results:

We present a 67-year-old female with a PMH of Sickle cell (HbSC) disease with retinopathy, splenectomy, cholecystectomy, atrial fibrillation on Eliquis, and hypertension who was initially admitted for management of vaso-occlusive crisis and presumed avascular necrosis of the right hip. Stroke code called for deteriorating mental status. CT Head and CTA of the Head & Neck were unremarkable but hyper-acute MRI of the brain was performed which showed multiple scattered embolic infarcts in both cerebral and cerebellar hemispheres. Patient was later intubated and monitored on LTM which did not show any signs of seizures or ictal-interictal abnormalities. Patient underwent erythocytopheresis with exchange of 8 Units. Pre and Post-HbS were 54% and 7% respectively. Given lack of improvement in exam, MRI Brain was repeated which showed additional foci of diffusion restriction as well as dispersed micro-hemorrhages concerning for CFES. Patient additionally underwent two sessions of plasmapheresis.

Conclusions:

This case illustrates that CFES should be suspected in patients with SCD when there is a change in mental status or neurologic deficit which is likely from bone marrow necrosis leading to fat globules being released into the systemic circulation. Early MRI is useful in establishing a diagnosis for prompt, timely treatment.