Fat Embolism Syndrome following minor trauma in Duchenne muscular dystrophy

Abstract 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...

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Bibliographic Details
Published in:Neuromuscular disorders : NMD 2012-12, Vol.22 (12), p.1035-1039
Main Authors: McAdam, Laura C, Rastogi, Anjali, Macleod, Kathleen, Douglas Biggar, W
Format: Article
Language:English
Subjects:
Acute respiratory distress syndrome
Adolescent
Adrenal Cortex Hormones - therapeutic use
Autopsy
Corticoids
Death
Duchenne muscular dystrophy
Duchenne's muscular dystrophy
Embolism
Embolism, Fat - diagnosis
Embolism, Fat - etiology
Fat Embolism Syndrome
Fatal Outcome
Female
Fractures
Humans
Ionizing radiation
Male
Muscular Dystrophy, Duchenne - complications
Muscular Dystrophy, Duchenne - diagnosis
Muscular Dystrophy, Duchenne - mortality
Neurology
neuromuscular system
Reminder
Survival
Trauma
Wounds and Injuries - complications
Young Adult
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Summary:Abstract 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.
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2012.07.010