Module 67, General Orthopaedist Level
This is a 5 year and 3 month old white female was the product of a
full-term normal spontaneous vaginal delivery with a diagnosis of CHARGE
syndrome (defects of the eyes, ears, heart, choanal atresia (primordal
oronasal opening into the brain, mental retardation, and genital hypoplasia).
She presented for evaluation of a webbed neck, left side worse than right.
The child has developmental delay and began walking at 4 years of age.
She had undergone previous surgery for strabismus and an atrial septal
defect. Physical exam revealed defects of the eyes and ears, the neck was
shortened and webbed (left worse than right). The back, shoulders and scapula
were elevated (left worse than right). The scapula was rotated down and
away from spine. Shoulder abduction was 110 degrees on right and 100 degrees
on left.
Question 67A
The congenital problem in this girl is a failure of segmentation which
occurred in the first trimester of development.
Question 67B
The
scapulae are connected together in the midline which prevents them from
moving laterally.
Question 67C
The
omovertebral bone on either side of the child is a major part of this deformity.
Question 67D
Sprengel's
deformity is much more common in girls and on the right side.
Question 67E
If
surgery is to be considered it should be done at a very early age, less
than age two years.
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