Toward a rational treatment of Chiari I malformation and syringomyelia

In patients with Chiari I malformation with and without associated syringomyelia, aberrant cerebrospinal fluid (CSF) dynamics and a spectrum of posterior fossa pathological findings are demonstrated. In this study, the authors test the validity of using prospective cardiac-gated phase-contrast cine-mode magnetic resonance (MR) imaging to define the malformation, delineate its pathophysiology, and assist in implementing a rational treatment plan. Eighty-five cases were prospectively analyzed using cine MR imaging. Sixty-five patients, adults and children, with symptomatic Chiari malformation, with and without syringomyelia, were surgically treated from 1990 to 1999. All patients underwent pre- and postoperative cine MR evaluation. Ten patients were treated after a previous surgical procedure had failed. To establish CSF flow characteristics and normative CSF profiles, 20 healthy volunteers were examined. Compared with normal volunteers, in Chiari I malformation patients with and without syringomyelia, uniformly abnormal craniocervical junction CSF flow profiles were revealed. After intradural exploration, nearly all patients with Chiari I malformation experienced clinical improvement and CSF flow profiles, paralleling those of normal volunteers, were shown. In all patients in whom treatment had failed, abnormal preoperative CSF flow profiles, which correlated with suspected physiological abnormalities and the pathological findings noted at reoperation, were demonstrated. Symptomatic Chiari I malformation is a dynamic process characterized by the impaction of the hindbrain in an abnormal posterior fossa. This compression obstructs the normal venting of CSF in and out of the craniocervical subarachnoid space, throughout the cardiac cycle. Therefore, decompression or enlargement of the posterior fossa to establish normal CSF pathways should be the primary goal of surgical intervention. Aberrant CSF flow appears to be only one aspect of the pathological condition found in patients with Chiari I malformation. Arachnoid scarring in the posterior fossa and selective vulnerability of the spinal cord may also be factors in the pathogenesis and maintenance of associated syringomyelia. Phase-contrast cine MR imaging is a useful tool in defining physiological and anatomical problems in patients with Chiari I and syringomyelia, and it can help guide an appropriate primary or salvage surgical therapy.