Abstract Details

Title Clinical and radiographic mismatch in fat embolism syndrome in hemoglobin SC disease

Topic General Neurology

Presentation(s) P5 - Poster Session 5 (5:30 PM-6:30 PM)

Poster/Presentation
Number 7-035

Objective NA

Background Fat embolism syndrome has been reported mostly in cases of long bone fractures and rarely in hemoglobinopathies due to bone marrow infarction. Nevertheless, it encompasses a wide range of multi-organ complications, including neurological, with devastating outcome. MRI findings of “starfield “pattern of innumerable restricted diffusion punctate foci with concordant decreased signal on ADC map have been reported.

Design/Methods We report a case of a 40-year old Nigerian male with hemoglobin SC with no previous crisis or complications who develops acute painful episodes likely vaso-occlusive pain minutes after fluorescein and indocyanine dye injection for retinal angiography status post retinal detachment repair, requiring PCA pump and hospitalization. Within 1 day of hospitalization, he became unresponsive with acute respiratory failure requiring intubation.

Results MRI brain at 7.5 hour was reported insignificant including susceptibility weighted images. EEG was consistent with diffuse encephalopathy. He developed anemia and severe thrombocytopenia. Patient was then transferred to our institution. Empiric antibiotics for possible CNS infection were initiated. CSF studies however were grossly unremarkable. Electrophoresis confirmed hemoglobin SC disease. Peripheral smear showed numerous immature nucleated RBCs. Bone marrow biopsy showed extensive fat necrosis. Repeat brain MRI on day 12 showed diffuse numerous punctate foci of restricted diffusion and numerous microhemorrhages and diffuse cerebral edema consistent with fat embolism syndrome. Despite RBC and multiple exchange transfusions, patient remained in persistent vegetative state.

Conclusions Successful outcomes have been reported in sickle cell disease patients with fat embolism syndrome who are treated with early aggressive exchange transfusions. Furthermore, susceptibility weighted imaging has been shown to be very sensitive in detecting a small amounts of deoxygenated blood products. However, our case demonstrates that the susceptibility weighted imaging can be normal hours after initial insult and the importance of repeating brain MRI in a timely manner and in the appropriate clinical setting.

Authors/Disclosures
Shahla Moghbel, MD PRESENTER	No disclosure on file
Arathi Nandyala, MD	Dr. Nandyala has nothing to disclose.
Steven E. Lo, MD (Medstar Georgetown University Hospital)	Dr. Lo has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Neurocrine.

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