Sickle cell disease related cerebral fat embolism: Characterizing the clinical syndrome

Abstract

To characterize the clinical syndrome of sickle cell disease (SCD) related cerebral fat embolism syndrome (CFES) SCD-related CFES results from bonemarrow embolism in the absence of trauma and except for a few single case reports, its clinico-imaging spectrum has not been fully characterized. Twelve SCD patients (6 women and 6 men, aged 28.4±9.9 years), were evaluated for neurological complications such as altered sensorium (n=12) and seizures (n=2) in the setting of acute chest syndrome, fever and no preceding trauma (n=12). Laboratory findings included high CRP (11 out of 11 tested) and thrombocytopenia (n=12), normal CSF findings (n=2 out of 2 tested). EEG showed non-specific diffuse slowing in 9 out of 10 cases and occasional left temporal spike discharges (n=1). Cerebral PET scan obtained in a single case showed globally reduced glucose uptake. Lipid laden macrophages were demonstrated in the broncho-alveolar lavage in two cases. CFES was diagnosed based on the characteristic finding of multiple cerebral microbleeds in the splenium and subcortical location on susceptibility weighted imaging (SWI). Our patients received exchange transfusion, antibiotics, ventilatory assistance (n=12) and anticonvulsant (n=2). One patient died, while the others survived with variable functional disability. In SCD patients presenting with acute chest syndrome, altered mental status and fever, the presence of thrombocytopenia and high CRP provides important clue to the possibility of CFES. The diagnosis is confirmed with SWI MRI that usually demonstrates multiple microbleeds in the splenium and subcortical areas. SCD-related CFES carries considerable short-term morbidity and occasional mortality.