Module 36, Pediatric Orthopaedist Level
A three month old girl presented for evaluation for disproportionate
short stature. The disproportionate stature was of the short limb type.
The head had normal configuration. There was a normal distribution of the
hair. The ears were thickened and inflamed. Epicanthic folds were not present.
The nose was broad at the root as well as in the mid-bridge. There was
clefting of the first part of the palate. She is diagnosed as diastrophic
dwarfism. By 13 months of age she developed an unstable kyphosis of the
cervical spine. By age 5 she had a very severe kyphoscoliosis of the thoracic
spine. At her most recent follow-up at age 9, she presented with increasing
hip and knee flexion contractures that altered her gait, the equinus contractures
of her ankles had returned and were very rigid.
Question 36A
The
primary treatment of the scoliosis is a plastic custom molded TLSO.
Question 36B
Spine
fusion at age 5 should be very limited to prevent stunting her growth further.
Question 36C
At age nine the ankle equinus is so contracted that it requires surgery.
You suggest a complete posterior ankle capsulotomy.
Question 36D
Hip
extension osteotomies are required to correct severe contractures to improve
walking ability
Question 36E
Total
hip replacement may be required in early adulthood to maintain ambulatory
ability.
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