Atlanto-Axial Dislocation in Down Syndrome: Report of Three Patients and Review of the Literature

Abstract

Atlanto-axial instability is a relatively frequent finding in a patient with Down syndrome. But there have been few reports of symptomatic atlanto-axial dislocation in Down syndrome. We report three cases of atlanto-axial dislocation associated with spinal cord compression. In addition, case reports and symptomtic atlanto-axial dislocation from the literature were analyzed. We conclude that an osseous malformation of the odontoid is the main cause of atlanto-axial instability and irreducibility of dislocation, and that appropriate surgical management, including posterior cervical spine fusion will in most instances result in a favorable outcome.