Chiari I malformation associated with atlanto-axial dislocation: focussing on the anterior cervico-medullary compression

Abstract

Chiari I malformation with atlantoaxial dislocation may cause both posterior and anterior cervicomedullary compression. We studied the clinicoradiological features and surgical outcome in patients having Chiari I malformation with atlantoaxial dislocation. Thirty-nine patients with Chiari I malformation with atlanto-axial dislocation underwent preoperative and follow-up neurological status assessment. In Chiari I malformation with reducible atlanto-axial dislocation (n = 11), a direct posterior stabilization was done. In Chiari I malformation with irreducible atlanto-axial dislocation (n = 28), a single stage transoral decompression with posterior stabilization and/or posterior decompression and duraplasty were done in 18 patients. In 10 patients, only posterior decompression and/or posterior stabilization was performed. Seven among the latter patients subsequently deteriorated and required transoral decompression. Comparison of mean neurological status scores of patients with Chiari I malformation with irreducible atlanto-axial dislocation who underwent single stage transoral decompression with posterior stabilization versus the posterior procedure alone was done using T-test and proportional significance also calculated. Patients with Chiari I malformation with atlanto-axial dislocation have a high incidence of long tract signs and sphincteric disturbances with a decrease in the mean foramen magnum diameter. The mean neurological status scores of patients with Chiari I malformation with irreducible atlanto-axial dislocation who underwent single stage transoral decompression with posterior stabilization were significantly better than those patients who underwent the posterior procedure alone. The latter patients also showed significant clinical improvement following transoral decompression. In the presence of Chiari I malformation with reducible atlanto-axial dislocation, reduction and stabilization of atlanto-axial dislocation resulted in neurological improvement. The follow up neurological status scores of these patients improved after surgical intervention even in the presence of poor preoperative grades. Patients with Chiari I malformation should be investigated for the presence of atlanto-axial dislocation. In case atlantoaxial dislocation coexists, priority must be given to relieving anterior cervicomedullary compression.