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68th Annual Meeting of the German Society of Neurosurgery (DGNC)
7th Joint Meeting with the British Neurosurgical Society (SBNS)

German Society of Neurosurgery (DGNC)

14 - 17 May 2017, Magdeburg

Evolution of Therapeutic Strategies for Syringomyelia

Meeting Abstract

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  • Jörg Klekamp - Christliches Krankenhaus, Zentrum Neurochirurgie, Quakenbrück, Deutschland

Deutsche Gesellschaft für Neurochirurgie. Society of British Neurological Surgeons. 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS). Magdeburg, 14.-17.05.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocDI.04.05

doi: 10.3205/17dgnc202, urn:nbn:de:0183-17dgnc2026

Published: June 9, 2017

© 2017 Klekamp.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at



Objective: Treatment of syringomyelia began at the end of the 19th century with surgical opening of the syrinx. In the first half of the 20th century different types of shunts were used to drain syrinx fluid to the subarachnoid space, peritoneal or pleural cavity. The introduction of decompression for Chiari I malformations in the 1960s marked the beginning of causative treatment for syringomyelia. With the advent of magnetic resonance imaging and recognition of an increasing number of pathologies leading to syringomyelia further strategies to treat the cause of syringomyelia were developed in the 1990s.

Methods: Since 1991 all patients with spinal cord pathologies were entered into a spinal cord data base. Between 1991 and 2015 a total ot 1535 patients with syringomyelia were encountered (follow up 47.1±55 months). Patients were examined on admission, after surgery, 3 months after discharge and yearly thereafter. Clinical data were analyzed with a scoring system for individual symptoms. Long-term results were determined by Kaplan-Meier statistics to calculate rates for progression-free survival.

Results: Syringomyelia was found to be caused by pathologies at the craniocervical junction in 604 patients (Chiari I malformations n = 543, foramen magnum arachnoiditis n = 32, Chiari II malformations n = 27, Chiari III malformations n = 2). For 931 patients pathologies of the spinal canal were responsible (nontraumatic arachnopathies n = 370, posttraumatic arachnopathies n = 163, intramedullary tumors n = 152, extramedullary intradural tumors n = 92, cervical disc diseases n = 70, tethered cord syndromes n = 69, extradural tumors n = 15). A total of 747 patients underwent surgery attempting to treat the cause of the syrinx without using syrinx shunts. For 83.7% causative treatment strategies resulted in progression-free survival rates of 70 – 90% for 10 years depending on the underlying pathology. Significantly worse long-term outcomes were achieved for patients with extensive arachnopathies related to subarachnoid hemorrhages or infections and for patients with posttraumatic syringomyelia related to an incomplete cord lesion. For this subgroup of 16.3%, an alternative strategy to treat syringomyelia has been employed draining cerebrospinal fluid from the subarachnoid space to the peritoneal cavity in 8 patients. For 15 patients with a complete cord lesion and ascending syringomyelia cordectomies were performed.

Conclusion: Syringomyelia may be caused by intramedullary tumors and pathologies either leading to disturbances of cerebrospinal fluid flow or cord tethering. For the great majority causative treatment strategies are available with good long-term results. Syrinx drainage procedures can be considered obsolete for first line treatment. For symptomatic therapy of syringomyelia drainage of cerebrospinal fluid from the subarachnoid space to the peritoneal cavity may be employed.