The diagnosis and management of occipital-atlantal and atlantoaxial instability in Down syndrome patients is a challenging problem in pediatric spine surgery. To date, no systematic review of this topic has been presented on this confusing and sometimes contentious issue. This topic review will focus on the biomechanical and radiographic foundations for which treatment recommendations in Down syndrome patients are made. In addition, otolaryngologic and anesthetic considerations in Down syndrome are also discussed, as well as advances in surgery that have made the operative fusion of these patients easier and safer.

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