Kyphoscoliosis in Williams Syndrome

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A 10-year-old girl with Williams syndrome (with characteristic facies and behavior, mental retardation, and growth disturbances) was seen with scoliosis, which despite attempted bracing, rapidly progressed to 95° and required surgical stabilization. Review of the entire literature on Williams syndrome revealed hallux vaigus and little-finger clinodactyly as the most commonly mentioned orthopaedic manifestations, with only brief mention of spinal deformity. As awareness of Williams syndrome increases, spine surgeons must be aware of possible rapidly progressive scollosis and kyphosis.

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