Neurospine.  2022 Sep;19(3):816-827. 10.14245/ns.2244332.166.

Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration

Affiliations
  • 1Division of Spine, Department of Neurosurgery, China International Neuroscience Institute (CHINA-INI), Xuanwu Hospital, Capital Medical University, Beijing, China
  • 2Research Center of Spine and Spinal Cord, Beijing Institute for Brain Disorders, Capital Medical University, Beijing, China
  • 3Lab of Spinal Cord Injury and Functional Reconstruction, China International Neuroscience Institute (CHINA-INI), Xuanwu Hospital, Capital Medical University, Beijing, China
  • 4Department of Neurosurgery, Pediatric Center, China International Neuroscience Institute (CHINA-INI), Xuanwu Hospital, Capital Medical University, Beijing, China

Abstract


Objective
The specific association between morphometric characteristics of the syrinx and the prognosis of Chiari malformation type I (CM-I) with syringomyelia following surgical procedure seems to have not been fully elaborated. This study focused on the preoperative clinical and radiologic parameters in CM-I patients with syringomyelia to find out the relationship between the patients’ clinical status and the phenotypes of the syrinx with surgical outcome.
Methods
A continuous series of pediatric and adult patients with CM-I and syringomyelia from a prospectively maintained database in a single center were included, and we explored the related factors affecting the prognosis following decompression surgery through retrospective analysis of clinical presentations, imaging characteristics, and the morphological features of syringomyelia, to provide a clinical reference for the treatment of syringomyelia.
Results
There were 28 pediatric patients (13.8%), and 174 adults (86.2%) included in our study. The average Chicago Chiari Outcome Scale score was 14.56 ± 1.78. The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration, ventral subarachnoid space at the foramen magnum, and with/without straightened cervical physio-curve.
Conclusion
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis.

Keyword

Syringomyelia; Chiari malformation type I; Posterior fossa decompression; Morphometric feature; Prognosis
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