Destruction or degeneration of gray and white matter adjacent to the central canal of the cervical spinal cord leads to cavitation and accumulation of fluid within the spinal cord. The precise pathogenesis is unclear, but many cases are associated with Arnold-Chiari malformation, in which there is displacement of the cerebellar tonsils, medulla, and fourth ventricle into the spinal canal, sometimes with accompanying meningomyelocele. In such circumstances, the cord cavity connects with and may merely represent a dilated central canal. In other cases, the cause of cavitation is less clear. There is a characteristic clinical picture, with segmental atrophy, areflexia, and loss of pain and temperature appreciation in a “cape” distribution, owing to the destruction of fibers crossing in front of the central canal (eFigure 24–15) in the mid-cervical spinal cord. Thoracic kyphoscoliosis is usually present. With progression, involvement of the long motor and sensory tracts occurs as well, so that a pyramidal and sensory deficit develops in the legs. Upward extension of the cavitation (syringobulbia) leads to dysfunction of the lower brainstem and thus to bulbar palsy, nystagmus, and sensory impairment over one or both sides of the face.
Syringomyelia (the presence of a cavity in the spinal cord due to breakdown of gliomatous new formations, presenting clinically with pain and paresthesias followed by muscular atrophy of the hands) involving the cervicothoracic portion of the cord. (Reproduced, with permission, from Waxman SG, deGroot J. Clinical Neuroanatomy, 22nd ed. Originally published by Appleton & Lange. Copyright © 1995 by The McGraw-Hill Companies, Inc.)
Syringomyelia, ie, cord cavitation, may also occur in association with an intramedullary tumor or following severe cord injury, and the cavity then does not communicate with the central canal.
In patients with Arnold-Chiari malformation, CT scans reveal a small posterior fossa and enlargement of the foramen magnum, along with other associated skeletal abnormalities at the base of the skull and upper cervical spine. MRI reveals the syrinx as well as the characteristic findings of the Arnold-Chiari malformation, including the caudal displacement of the fourth ventricle and herniation of the cerebellar tonsils through the foramen magnum. Focal cord enlargement is found at myelography or by MRI in patients with cavitation related to past injury or intramedullary neoplasms.
Treatment of Arnold-Chiari malformation with associated syringomyelia is by suboccipital craniectomy and upper cervical laminectomy, with the aim of decompressing the malformation at the foramen magnum. The cord cavity should be drained and, if necessary, an outlet for the fourth ventricle can be made. In cavitation associated with intramedullary tumor, treatment is surgical, but radiation therapy may be necessary if complete removal is not possible. Posttraumatic syringomyelia is also treated surgically if it leads to increasing neurologic deficits or to intolerable pain.