Diagnostic and Management of Traumatic Syringomyelia at the Craniocervical Junction

Introduction: Trauma to the craniocervical junction or the cervical spine can lead to arachnopathic adhesions of neural tissue with subsequent development of cervical or bulbar syringomyelia concomitant with a dramatic deterioration of clinical symptoms. The diagnostic procedures and surgical management in these delicate cases is described and risks and benefits are outlined.

Methods: Seven patients with severe injuries to either the upper cervical spine or the craniocervical junction were treated at the Department of Neurosurgery, University of Tübingen, within a 2-year period. They complained about a progressive loss of neurological function within a short period of time, due to the enlargement of cervical and bulbar syringomyelia. All patients underwent a standardized workup including neurological assessment; electrophysiological measurements include routine SEP and MEP for all extremities as well as the evaluation of the silent periods for detection of alteration in Aδ-pain fibers. MRI diagnostic then became a central part of the diagnostic procedures: in addition to routine acquisitions with FLAIR, T1-/T2- weighted images and contrast-enhanced series, we applied sagittal cardiac-gated sequences (CINE) for visualization of CSF pulsations and axial 3D-constructive interference in steady state (CISS) sequences, to detect focal arachnoid adhesions. If on MRI imaging a suspicious region was detected, axial postmyelographic CT scans were performed to obtain highest visibility of subarachnoid scar tissue formation. An indication for neurosurgical intervention emerged if a focal adhesion due to arachnopathic scar tissue was detected as the underlying cause of CSF obstruction forming the syringomyelia. The operation aimed to decompress the subarachnoid space forming a new unobstructed CSF pathway, including an enlarging duroplasty. In cases of a stable and long-lasting complete paraplegia, a forced myelolysis was performed to release pressure of the syrinx cavity in order to save the patient from bulbar symptoms.

Results: All treated patients show a collapse of the syrinx, some within days after surgery. Neurological symptoms did improve in all cases, but in most cases longstanding neurological deficits remained stable.

Conclusion: Aggravation of deficits due to the natural history of the disease can be stopped. We demonstrate the diagnostic and operative management in these patients, emphasizing the necessity for meticulous spinal trauma detection.