Examination: 5-Year-old with Increasing Spinal Deformity

Examination
Clinical evaluation showed a tall, thin, 5-year-old male with significant left convex kyphoscoliosis, lumbar hyperlordosis, and generalized weakness, especially around the pelvic and shoulder girdles (Figure 1A-1E).

Figure 1A

Figure 1B

Figure 1C

Figure 1D

Figure 1E

The spinal deformity was noted to be quite flexible with underarm suspension of the trunk. He had a +ve Gower's sign, hyperextensible joints, his knees showed 3+ laxity in all planes, and his patellae were easily dislocatable, although there was little discomfort associated with provoked dislocation.

When wearing braces (modified knee "cages" with outside hinges designed to prevent excessive varus-valgus and anterior-posterior movement), the patient was able to ambulate, but had become cautious due to the unbalanced forward position of the trunk combined with his hip girdle weakness.