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Six cases of relatively uncommon symptomatic atlantoaxial subluxation in Down's syndrome were treated surgically. In all cases, various congenital osseous anomalies were found. Marked myelopathy was noted in four cases of ossiculum terminale and mild myelopathy in one case of anteroposterior spondyloschisis. Five cases were treated successfully with posterior arthrodesis of C1 to C2 and one from the occiput to C2. Excellent bony union and overall improvement in the patients' symptoms was achieved, with a follow-up period ranging from 11 months to three years and nine months. In cases of symptomatic atlantoaxial subluxation of Down's syndrome, where the spinal cord is at risk, early surgical stabilization is the treatment of choice.
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