gms | German Medical Science

Introduction: Minimal spinal trauma as the cause of a Syringomyelia is commonly neglected in the management of this chronic and potentially disabling disease. Neurological deficits occurring late and are therefore most often not debated as the cause of syrinx development. However the restoration of adequate CSF-circulation can lead to cessation of disease progression. We present an optimized preoperative diagnostic protocol for syringomyelia patients in order to detect the underlying pathology.

Material and methods: All Syringomyelia patients transferred to our neurosurgical department undergo a standardized workup including neurological assessment with a meticulous history taking of the patient, to detect any spinal trauma occurred. Electrophysiological measurements include routine SEP and MEP for all extremities as well as silent-period testing for detection of alteration in A? pain fibres. MRI diagnostic then comprised a central part of the diagnostic procedures: Beside routine acquisitions with FLAIR, T1-/T2 weighted images and contrast enhanced series we apply sagittal cardiac-gated sequences (CINE) for visualisation of CSF-pulsations and axial 3D - constructive - interference- in steady - state (CISS) sequences, to detect focal arachnoid webs. If on MRI imaging a suspicious region is detected, axial postmyelographic CT scans are performed to obtain highest visibility of subarachnoid scar tissue formation. An indication for neurosurgical intervention emerges if a focal adhesion due to arachnopathic scar tissue is detected as the underlying cause of CSF-obstruction forming the Syringomyelia. The operation aims to decompress the subarachnoid space forming a new unobstructed CSF pathway, including an enlarging duroplasty.

Results: 75 patients with syringomyelia prospectively underwent the diagnostic protocol. With the expanded electrophysiological workup, a differentiation between hydromyelia (dilated central canal) and syringomyelia with alteration of crossing spinal cord tracts can be made. The sensitivities/specificities of the silent-periods for several syringomyelia symptoms (e.g. dysaesthesia, pain) are significantly higher than of SEP/MEP recordings. With the specialized MRI-diagnostics 24% more patients could be diagnosed with the syringomyelia-causing pathology: A spinal arachnoid web, cyst or scar developed through a former spinal trauma. All treated patients showed an immediate collapse of the syrinx, neurological symptoms did improve; however in most cases long-standing neurological deficits remain stable.

Conclusion: Syringomyelia should be seen as a symptom of an underlying arachnoid process, most often caused by a minor spinal trauma with subsequent arachnoid scar formation causing CSF flow obstruction. Neurosurgical detection and elimination of this obstruction lead to a free CSF passage and can stop the progredient course of the disease. Aggravation of deficits due to the natural history of the disease can be stopped with restoration of adequate CSF flow. We want to demonstrate the diagnostic and operative management in these patients exemplarily, emphasizing the necessity for meticulous spinal trauma detection.