Clinical and neuroimaging features of Chiari type I malformations with and without associated syringomyelia

The clinical and neuroimaging characteristics of 22 consecutive patients with Chiari type I malformations were evaluated to investigate the pathogenesis of syrinx formation. All patients underwent magnetic resonance imaging and x-ray tomography before surgery. The electric manometric Queckenstedt test was performed on 16 patients pre- and postoperatively. Syringomyelia was present in 17 patients and absent in five patients. All patients without syringomyelia suffered from foramen magnum compression syndrome, with a wider basal angle, more acute clivo-axial angle, shorter clivus, and more prominent tonsillar ectopia than patients with syringomyelia. Low brain stem position, basilar impression, and beaking of the cervicomedullary junction were also more prominent in patients without syringomyelia. Marked to complete block with the neck in flexed position by Queckenstedt test was present in all patients except one. Patients with Chiari malformation not associated with syringomyelia have more pronounced compression of the brain stem at the foramen magnum. Therefore, despite a block of the cerebrospinal fluid pathway at the foramen magnum, syrinx formation may be prevented by severe compression.