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Published on: December 5, 2019
Fatal Fat Embolism Syndrome Without Recognized Fracture in Patients With Duchenne Muscular Dystrophy: Two Case
Cameron A Wade1, Chet Villa1, Larry B Goldstein2
1Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; University of Cincinnati College of Medicine, Cincinnati, Ohio.
Background:
Fat embolism syndrome (FES) is an uncommon cause of sudden death in patients with Duchenne muscular dystrophy (DMD), typically following identifiable fractures.
Methods:
We present two cases of sudden fatal FES in the setting of DMD without clinically recognized trauma or fracture. The first was a 7-year-old boy who had autism spectrum disorder and who also had sudden death after collapsing at home following a walk with no recognized trauma. The second was a 23-year-old man who developed sudden respiratory failure without preceding illness or injury that progressed to shock and subsequent death.
Results:
Autopsy in both cases revealed extensive fat emboli in the microvasculature in multiple organs.
Conclusions:
These cases underscore the risk of FES in patients with DMD without apparent preceding injury.
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