Fat Embolism Syndrome following minor trauma in Duchenne muscular dystrophy.

Neuromuscul Disord

Holland Bloorview Kids Rehabilitation Hospital, Toronto, Canada; Hospital for Sick Children, Toronto, Canada.

Published: December 2012

We describe five patients with Duchenne muscular dystrophy who presented with acute neurologic and respiratory symptoms following minor trauma. Four of the five deteriorated rapidly and died within 36 h after falling. X-rays for fractures were negative. Four of the five patients were taking corticosteroids daily. All five patients fulfilled the clinical criteria for Fat Embolism Syndrome. Autopsy findings were consistent with fat embolism in two cases. Fat Embolism Syndrome needs to be considered in patients with Duchenne muscular dystrophy following minor trauma even without fractures. Early recognition of Fat Embolism Syndrome and aggressive resuscitation are important to improve survival. This report serves as an important reminder that seatbelts need to be used at all times.

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Source
http://dx.doi.org/10.1016/j.nmd.2012.07.010DOI Listing

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