The management of atlanto-axial subluxation with neurologic involvement in Down's syndrome: a report of two cases and review of the literature.

A review of 5 cases in the literature and our 2 additional cases of atlanto-axial sublux-ation in Down's Syndrome with associated neurologic deterioration suggests that posterior stabilization and fusion should be carried out initially. Posterior decompression alone, with excision of the posterior arch of C-1, may further increase atlantoaxial instability and contribute to neurologic deterioration. Lateral radiographs in flexion and extension and contrast studies are recommended to assess the reducibility of the lesion and the site of neural impingement. It is further recommended that if significant neurologic deterioration persists or worsens, an oral transpharyngeal odontoidectomy should be considered for cord decompression.