Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases

Neurosurgery. 2009 Feb;64(2):308-15; discussion 315. doi: 10.1227/01.NEU.0000336768.95044.80.


Objective: To determine the long-term outcome of surgically treated Chiari-related syringomyelia.

Methods: The medical charts of 157 consecutive surgically treated patients with Chiari-related syringomyelia were retrospectively analyzed. Factors predicting outcome, either clinical or radiological, are discussed, and our results are compared with those of other large series in the literature.

Results: The study included 74 men and 83 women (age range, 16-75 years; mean age at surgery, 38.3 years). Pain and sensory disturbance were the most frequent initial symptoms. The average duration of preoperative symptoms was 8.2 years. The follow-up period ranged from 82 to 204 months (median, 88 months). At the end of the study, 99 patients (63.06%) had improved, 48 (30.58%) had stabilized, 9 (5.73%) had worsened, and 1 (0.63%) had died during the postoperative period. Factors predicting improvement or stabilization were young age at the time of surgery and clinical signs of paroxysmal intracranial hypertension. Factors associated with a poor outcome were older age at the time of surgery, arachnoiditis, and a clinical feature of long-tract impairment syndrome. The presence of arachnoiditis or of basilar invagination was associated with poor clinical presentation (P = 0.05 and 0.0001, respectively). The extent of the cyst on postoperative magnetic resonance imaging was a predictor of poor clinical outcome (P = 0.002).

Conclusion: Our results confirmed that surgery is an effective and safe treatment of Chiari-related syringomyelia, with a 90% chance of long-term stabilization or improvement on average. Surgery should be proposed as soon as possible in patients with clearly progressing clinical features.

Publication types

  • Meta-Analysis

MeSH terms

  • Adolescent
  • Adult
  • Arnold-Chiari Malformation / epidemiology*
  • Arnold-Chiari Malformation / surgery*
  • Comorbidity
  • Female
  • Follow-Up Studies
  • France / epidemiology
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Risk Assessment / methods*
  • Risk Factors
  • Syringomyelia / epidemiology*
  • Syringomyelia / surgery*
  • Treatment Outcome
  • Young Adult