Objective Idiopathic syringomyelia (IS) associated with occult arachnoid pathology is a relatively rare condition characterized by a subtle onset, atypical clinical manifestations, and significant diagnostic and therapeutic challenges. This study aims to evaluate the radiographic and clinicopathological features of IS to improve surgical management and patient outcomes.
Methods In this study, clinical and radiologic data were retrospectively extracted from a single-center syringomyelia database (N=1,039) spanning December 2020 to March 2025. Among these, 15 patients diagnosed with IS underwent preoperative magnetic resonance imaging and myelography to identify the responsible spinal segments precisely. Comprehensive perioperative assessments and clinical outcomes were collected. During surgery, the subarachnoid space (SAS) was thoroughly explored, with complete removal of thickened and adherent arachnoid tissue to restore normal cerebrospinal fluid (CSF) circulation. Additionally, clinical data, pathological features, and surgical outcomes of IS were compared to those of posttraumatic delayed syringomyelia (PTDS) to evaluate potential differences.
Results In this series, all patients underwent preoperative myelography, revealing varying degrees of SAS obstruction. For IS cases that received precise and comprehensive arachnoid lysis, overall postoperative outcomes were favorable. Intraoperative pathology confirmed that all IS cases were characterized by noninfectious, nonacute inflammation. The preoperative maximal syrinx/cord ratio averaged 0.70±0.07 (range, 0.54–0.88), while the syrinx resolution rate varied from 12.2% to 100%, with a mean improvement of 29.6%. Patients with PTDS exhibited a relatively higher incidence of hypesthesia and a greater syrinx tension index. However, no significant differences were observed between IS and PTDS in terms of syrinx length, deviation, or location. Notably, the IS group demonstrated significantly better postoperative syrinx resolution and improvement in syringomyelia-related symptoms compared to the PTDS group.
Conclusion While both IS and PTDS share a common underlying mechanism of arachnoid adhesions, they differ significantly in pathological features, treatment approaches, and clinical outcomes. In cases of IS, thorough spinal arachnoid lysis at the affected segment could restore normal spinal cord pulsation and CSF circulation, leading to effective syrinx resolution and a favorable long-term prognosis.
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Objective Syringomyelia is a common central nervous system disease characterized by the dilation of the central canal (CC). Regarding the pathogenesis of syringomyelia, cerebrospinal fluid (CSF) circulation obstruction in the subarachnoid space (SAS) of the spinal cord has been widely accepted. However, clinical and animal studies on obstructing the CSF in SAS failed to form syringomyelia, challenging the theory of SAS obstruction. The precise pathogenesis remains unknown.
Methods We utilized an extradural compression rat model to investigate the pathogenesis underlying syringomyelia. Magnetic resonance imaging enabled detection of syringomyelia formation. To assess CSF flow within the SAS, Evans blue was infused into the cisterna magna. Histological analysis allowed morphological examination of the CC. Furthermore, CSF flow through the CC was traced using Ovalbumin Alexa-Flour 647 conjugate (OAF-647). Scanning electron microscopy (SEM) enabled visualization of ependymal cilia.
Results The findings showed that the dura mater below the compression segment exhibited lighter coloration relative to the region above the compression, indicative of partial obstruction within the SAS. However, the degree of SAS occlusion did not significantly differ between syringomyelia (SM-Y group) and those without (SM-N group). Intriguingly, hematoxylin and eosin staining and CSF tracing revealed occlusion of the CC accompanied by reduced CSF flow in the SM-Y group compared to SM-N and control groups. SEM images uncovered impairment of ependymal cilia inside the syringomyelia.
Conclusion CC occlusion may represent a physiological prerequisite for syringomyelia formation, while SAS obstruction serves to initiate disease onset. The impairment of ependymal cilia appears to facilitate progression of syringomyelia.
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Objective Combined with different configuration types of syringomyelia, to analyze the correlation between syrinx resolution and changes in cervical sagittal alignment following Foramen magnum and Magendie dredging (FMMD) for syringomyelia associated with Chiari I malformation (CM-I), and to further explore the respective relationship with clinical outcome.
Methods A consecutive series of 127 patients with CM-I and syringomyelia who underwent FMMD in our center met the inclusion criteria of this study. Their clinical records and radiologic data were retrospectively reviewed. The Japanese Orthopedic Association (JOA) scoring system and the Chicago Chiari Outcome Scale (CCOS) were used to evaluate the surgical efficacy. The phenotypes of syringomyelia and the clinical characteristics of the patients were analyzed according to grouping by cervical curvature at baseline.
Results The preoperative straight or kyphotic cervical alignment is more common in the moniliform syrinx. After surgery, the syrinx resolution and cervical sagittal realignment in the moniliform group are more obvious, and the corresponding prognosis is relatively better. Spearman correlation analysis showed that the ΔS/C ratio (the change ratio of syrinx/cord) was positively correlated with the CCOS (p = 0.001, r = 0.897) and ΔC2–7A (the change of lower cervical angle) (p = 0.002, r = 0.560). There was also a correlation between the ΔJOA score (the change rate of the JOA score) and ΔC2–7A (p = 0.012, r = 0.467).
Conclusion After decompression surgery, syrinx resolution may coexist with the changes in the subaxial lordosis angle, especially for syrinx in moniliform type, and the relationship between syrinx resolution and cervical sagittal realignment might be valuable for evaluating the surgical outcome.
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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.
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