Syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature.

Abstract

Idiopathic syringomyelia, which is not associated with any definite pathogenic lesions, has been treated mainly by shunting of the syrinx and rarely by craniocervical decompression. The authors report four cases of syringomyelia thought to be idiopathic syringomyelia but treated by craniocervical decompression with favorable results. Syringomyelia was present without hindbrain herniation. In such cases, the subarachnoid space anterior to the brainstem at the level of the foramen magnum is usually open but the cisterna magna is impacted by the tonsils, a condition the authors term "tight cisterna magna." All patients underwent foramen magnum decompression and C-1 laminectomy, and the outer layer of the dura was peeled off. Further intradural exploration was performed when outflow of cerebrospinal fluid (CSF) from the fourth ventricle was deemed to be insufficient. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in three patients, and a reduction in ventricle size was shown in two. Syringomyelia associated with tight cisterna magna should not be classified as idiopathic syringomyelia; rather, it belongs to the category of organic syringomyelia such as Chiari malformation. A possible pathogenesis of cavitation is obstruction of the CSF outflow from the foramen of Magendie, and the cavity may be a communicating dilation of the central canal. Ventricular dilation may depend on the extent to which CSF drainage is impaired from the foramina of Luschka. These cavities may respond to craniocervical decompression if it results in sufficient CSF outflow from the foramen of Magendie, even in cases with concomitant hydrocephalus.