Module 2, Pediatric Orthopaedist Level
This 16 year old black male presented with the chief complaint of left
scapular winging and weakness of the left arm. Patient also complained
of the inability to abduct arms beyond the horizontal position. He had
also notice increasing atrophy of the left arm. He was active in sports
and played football but was unable to play basketball. Facial characteristics
included a transverse smile, inability to purse lips or whistle, and inability
to close his eyes completely. Active abduction of the shoulders was approximately
90 degrees with a normal passive ROM. Motor strength of (L) UE was 4/5
except deltoid which as 5/5. Both scapulae were elevated and medially rotated
with marked winging noted with attempted abduction. EMG and NCS revealed
a myopathic process. There is no family history of similar conditions,
however the diagnosis is felt to be fascioscapulohumeral dystrophy (FSH).
Question 2A
The
shoulder girdle weakness is expected rapidly progress with no current treatment
options for this boy.
Question 2B
Careful
monitoring of scoliosis is recommended with a high risk of developing progressive
kyphoscoliosis.
Question 2C
Scapulothoracic
fusion would improve shoulder abduction.
Question 2D
This
boy can not whistle.
Question 2E
FSH is usually not diagnosed until the teenage years unless it is seen
in an affected family member.
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