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Chiari Malformation in Adults: A Review of 40 Cases

Published online by Cambridge University Press:  18 September 2015

David D.R. Eisenstat ,
Mark Bernstein ,
J.F. Ross Fleming ,
R.G. Vanderlinden  and
Hart Schutz
David D.R. Eisenstat
Affiliation:
Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto
Mark Bernstein*
Affiliation:
Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto
J.F. Ross Fleming
Affiliation:
Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto
R.G. Vanderlinden
Affiliation:
Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto
Hart Schutz
Affiliation:
Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto
*
25 Leonard Ave., Ste. 211, Toronto, Ontario, Canada M5T 2R2
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Abstract:

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Forty adult patients (average age 40 years), with the clinical and radiological features of the Chiari malformations, were seen at the Toronto Western Hospital between 1967 and 1984. Surgical confirmation of the diagnosis was obtained in 32 cases; of these, 23 were classified as Chiari I malformation while 9 fulfilled the anatomic criteria of Chiari II. The patient population consisted of 22 males and 18 females. Common presenting symptoms included head and neck pain (60%), sensory complaints (60%), upper extremity weakness (42%), and gait disturbance (40%). Neurological findings included signs of central cord dysfunction (73%), long-tract motor and/or sensory findings (58%), brainstem signs (38%), cerebellar dysfunction (18%), and increased intracranial pressure (15%). The majority of patients underwent myelography with or without computed tomography of the cervical-medullary junction. Two recent patients had 0.15T MRI scans which helped demonstrate an intramedullary syrinx. Thirty-three patients underwent 47 operative procedures (discounting spinal fusion and CSF shunt revisions). Open surgical management was performed in 32 patients, with CSF shunting along in one patient. Five patients (15%) incurred surgical complications within a six week postoperative period. Follow-up to date, ranges from one month to 11 years. In the 33 surgically treated patients, 18 are improved (55%), 10 are neurologically stable (30%), and five have worsened clinically (15%), including one death. Based on this study it appears that the Chiari II malformation maybe more common in adults than previously recognized. Surgical intervention has a favourable outcome in the majority of patients but a significant proportion continue to deteriorate.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 13 , Issue 3 , August 1986 , pp. 221 - 228
Copyright
Copyright © Canadian Neurological Sciences Federation 1986

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