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Pediatr Neurol. 2016 Oct;63:76-79. doi: 10.1016/j.pediatrneurol.2016.05.013. Epub 2016 Jun 8.

Recurrent Fat Embolic Strokes in a Patient With Duchenne Muscular Dystrophy With Long Bone Fractures and a Patent Foramen Ovale.

Author information

1
The Heart Center, Nationwide Children's Hospital, Columbus, Ohio. Electronic address: Christopher.Bugnitz@nationwidechildrens.org.
2
The Heart Center, Nationwide Children's Hospital, Columbus, Ohio.

Abstract

BACKGROUND:

Individuals with Duchenne muscular dystrophy have an increased risk of long bone fractures. Such fractures are sometimes associated with brain dysfunction due to fat embolism syndrome, although this syndrome has seldom been documented in muscular dystrophy patients.

PATIENT DESCRIPTION:

We describe a child with Duchenne muscular dystrophy who developed fat embolism syndrome with neurological dysfunction following multiple long bone fractures. He experienced recurrent cerebral infarctions that probably resulted from embolization through a patent foramen ovale. The patent foramen ovale was closed by an occluder device in the cardiac catheterization laboratory, and he did not experience further infarctions.

CONCLUSIONS:

Fat embolism with ischemic cerebral infarction can occur in individuals with Duchenne muscular dystrophy following long bone fractures. In this setting it is important to identify and close atrial level shunts in order to prevent additional infarctions.

KEYWORDS:

Duchenne muscular dystrophy; atrial septal defect; cardiomyopathy; fat embolism syndrome; patent foramen ovale; stroke

[Indexed for MEDLINE]

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