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 The anterior controllable antedisplacement and fusion (ACAF) technique is a new procedure for the treatment of cervical ossification of the posterior longitudinal ligament (OPLL) that requires management of the disc adjacent to the ossification. This study describes a novel technique to reduce the number of fixed segments, namely, the “Klotski technique.” The efficacy of ACAF using the Klotski technique was compared with that of anterior cervical corpectomy and fusion (ACCF) in the treatment of OPLL with en bloc type dural ossification (DO).
Methods The clinical data of 25 patients with severe OPLL and en bloc type DO who were treated by the ACAF Klotski technique or ACCF at our hospital from January 2020 to January 2022 were retrospectively analyzed. In the Klotski technique, the number of segments fused within the OPLL is limited. The antedisplacement space was designed according to the shape of the vertebrae-OPLL-DO complex (VODC). Then, the entire VODC was antedisplaced as in Klotski. Neurological function and image examination were assessed preoperatively and postoperatively. Complications associated with surgery were recorded.
Results Patients were followed up for 24–36 months. There were 11 patients who were treated with ACAF and 14 patients who were treated with ACCF. At 2 weeks after surgery, the incidence of neurological deterioration was 21.4% (3 of 14) in the ACCF group and 9.1%
(1 of 11) in the ACAF group. The incidence of intraoperative cerebrospinal fluid leakage (CFL) was 35.7% (5 of 14) in the ACCF group and 9.1% (1 of 11) in the ACAF group. The postoperative follow-up JOA scores of the patients in both groups were significantly better than their preoperative JOA scores (p<0.05).
Conclusion The Klotski technique for ACAF is a good option for the treatment of patients with en bloc type OPLL-DO, as it limits the number of fused segments, has a low incidence of CFL and neurologic deficits and is associated with good neurological recovery.
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Objective Primary spinal cord glioblastoma (PSCGBM) is a rare malignancy with a poor prognosis. To date, no prognostic nomogram for this rare disease was established. Hence, we aimed to develop a nomogram to predict overall survival (OS) of PSCGBM.
Methods Clinical data of patients with PSCGBM was retrospectively collected from the neurosurgery department of Soochow University Affiliated Second Hospital and the Surveillance Epidemiology and End Results database. Information including age, sex, race, tumor extension, extent of resection, adjuvant treatment, marital status, income, year of diagnosis and months from diagnosis to treatment were recorded. Univariate and multivariate Cox regression analyses were used to identify independent prognostic factors for PSCGBM. A nomogram was constructed to predict 1-year, 1.5-year, and 2-year OS of PSCGBM.
Results A total of 132 patients were included. The 1-year, 1.5-year, and 2-year OS were 45.5%, 29.5%, and 18.9%, respectively. Four variables: age groups, tumor extension, extent of resection, and adjuvant therapy, were identified as independent prognostic factors. The nomogram showed robust discrimination with a C-index value for the prediction of 1-year OS, 1.5-year OS, and 2-year of 0.71 (95% confidence interval [CI], 0.61–0.70), 0.72 (95% CI, 0.62–0.70), and 0.70 (95% CI, 0.61–0.70), respectively. The calibration curves exhibited high consistencies between the predicted and observed survival probability in this cohort.
Conclusion We have developed and internally validated a nomogram for predicting the survival outcome of PSCGBM for the first time. The nomogram has the potential to assist clinicians in making individualized predictions of survival outcome of PSCGBM.
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Objective To investigate the developmental defects caused by knockdown of best1 gene in zebrafish as a model for a subtype of craniovertebral junction (CVJ) malformation.
Methods Two antisense morpholinos (MOs) were designed targeting zebrafish best1 to block translation (ATG-MO) or to disrupt splicing (I3E4-MO). MOs were microinjected into fertilized one-cell embryos. Efficacy of splicing MO was confirmed by reverse transcription-polymerase chain reaction. Phenotypes were analyzed and quantified by microscopy at multiple developmental stages. Neuronal outgrowth was assessed in transgenic zebrafish expressing green fluorescent protein in neurons. Skeletal ossification was visualized by Calcein staining.
Results Knockdown of best1 resulted in zebrafish embryos with shorter body length, curved axis, low survival rate, microcephaly, reduced eye size, smaller head and brain, impaired neuronal outgrowth, and reduced ossification of craniofacial and vertebral bone.
Conclusion Best1 gene plays critical roles in ophthalmologic, neurological and skeletal development in zebrafish. A patient with a premature stop codon in BEST1 gene exhibited similar phenotypes, implying a subtype of CVJ malformation.
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Objective Surgical procedures for patients with posttraumatic syringomyelia (PTS) remain controversial. Until now, there have been no effective quantitative evaluation methods to assist in selecting appropriate surgical plans before surgery.
Methods We consecutively enrolled PTS patients (arachnoid lysis group, n = 42; shunting group, n = 14) from 2003 to 2023. Additionally, 19 intrathecal anesthesia patients were included in the control group. All patients with PTS underwent physical and neurological examinations and spinal magnetic resonance imaging preoperatively, 3–12 months postoperatively and during the last follow-up. Preoperative lumbar puncture was performed and blood-spinal cord barrier disruption was detected by quotient of albumin (Qalb, cerebrospinal fluid/serum).
Results The ages (p = 0.324) and sex (p = 0.065) of the PTS and control groups did not differ significantly. There were also no significant differences in age (p = 0.216), routine blood data and prognosis (p = 0.399) between the arachnoid lysis and shunting groups. But the QAlb level of PTS patients was significantly higher than that of the control group (p < 0.001), and the shunting group had a significantly higher QAlb (p < 0.001) than the arachnoid lysis group. A high preoperative QAlb (odds ratio, 1.091; 95% confidence interval, 1.004–1.187; p = 0.041) was identified as the predictive factor for the shunting procedure, with the receiver operating characteristic curve showing 100% specificity and 80.95% sensitivity for patients with a QAlb > 12.67.
Conclusion Preoperative QAlb is a significant predictive factor for the types of surgery. For PTS patients with a QAlb > 12.67, shunting represents the final recourse, necessitating the exploration and development of novel treatments for these patients.
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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 Although cerebrospinal fluid (CSF)-based liquid biopsy was proved to be practical in molecular analysis of intracranial gliomas, liquid biopsy of primary intramedullary astrocytoma was rarely reported. Given the distinct genomic profiles between primary intramedullary glioma and intracranial astrocytoma, whether the feasibility of CSF-based molecular analysis of intracranial gliomas can be replicated in primary spinal cord astrocytoma needs to be investigated. The aim of this pilot study is to evaluate the feasibility of molecular analysis of primary intramedullary astrocytoma through sequencing CSF-derived circulating tumor DNA (ctDNA).
Methods Two grade IV diffuse midline gliomas, 1 grade II, and 1 grade I astrocytoma were included. Intraoperative collection of peripheral blood and CSF samples was conducted, along with postoperative collection of matched tumor tissues. A panel covering the 1,021 most common driver genes of solid tumors was used for targeted DNA sequencing.
Results CSF-derived ctDNA was detected in 3 CSF samples (2 grade IV diffuse midline gliomas and 1 grade I astrocytoma), 5 mutations were found in both tumor tissues and CSF samples, while 11 mutations and 20 mutations were detected exclusively in tumor tissues and CSF samples, respectively. Importantly, hotspot genetic alterations, including H3F3AK28M, TP53, and ATRX, were identified in CSF and the average mutant allele frequency was often higher in CSF than in tumor tissues.
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Objective The surgical management of basilar invagination without atlantoaxial dislocation (type B basilar invagination) remains controversial. Hence, we have reported the use of posterior intra-articular C1–2 facet distraction, fixation, and cantilever technique versus foramen magnum decompression in treating type B basilar invagination as well as the results and surgical indications for this procedure.
Methods This was a single-center retrospective cohort study. Fifty-four patients who underwent intra-articular distraction, fixation, and cantilever reduction (experimental group) and foramen magnum decompression (control group) were enrolled in this study. Distance from odontoid tip to Chamberlain’s line, clivus-canal angle, cervicomedullary angle, craniovertebral junction (CVJ) triangle area, width of subarachnoid space and syrinx were used for radiographic assessment. Japanese Orthopedic Association (JOA) scores and 12-item Short Form health survey (SF-12) scores were used for clinical assessment.
Results All patients in the experimental group had a better reduction of basilar invagination and better relief of pressure on nerves. JOA scores and SF-12 scores also had better improvements in the experimental group postoperation. SF-12 score improvement was associated with preoperative CVJ triangle area (Pearson index, 0.515; p = 0.004), cutoff value of 2.00 cm2 indicating the surgical indication of our technique. No severe complications or infections occurred.
Conclusion Posterior intra-articular C1–2 facet distraction, fixation, and cantilever reduction technique is an effective treatment for type B basilar invagination. As various factors involved, other treatment strategies should also be investigated.
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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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Objective This study aimed to investigate the outcome of using 3-dimensional (3D)-printed prostheses to reconstruct a cervical lateral mass to maintain cervical stability.
Methods We retrospectively analyzed data of 7 patients who underwent cervical lateral mass reconstruction using a 3D-printed prosthesis, comprising axial and subaxial lateral mass reconstruction in 2 and 5 patients, respectively. Bilateral mass was reconstructed in 1 patient and unilateral mass in the remaining 6 patients.
Results Using a 3D-printed lateral mass prosthesis, internal fixation was stable for all 7 patients postoperatively. No implant-related complications such as prosthesis loosening, displacement, and compression were observed at the last follow-up.
Conclusion Reconstruction of the lateral mass structure is beneficial in restoring load transfer in the cervical spine under physiological conditions. A 3D-printed prosthesis can be considered a good option for reconstruction of the lateral mass as fusion was achieved, with no subsequent complications observed.
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Objective This study aimed to illustrate the features of fistula location distribution, surgical strategies, and outcomes in spinal epidural meningeal cysts (SEMCs).
Methods The authors searched and reviewed the medical records for cases of SEMCs. Imaging features, operative reports, and media were reviewed to accurately describe the surgical techniques employed. We recorded the level and laterality of the fistula according to the operative report and the media. Consistency analysis was performed on the dominant laterality of the cyst on preoperative axial magnetic resonance imaging and laterality of the fistula in the operative media or report. When cyst and fistula lateralities were the same, they were considered consistent. Finally, the Japanese Orthopedic Association (JOA) score was used to obtain patient-reported results at each follow-up.
Results Thirty patients with SEMCs were identified. Fistula repair was performed in all patients. Two patients experienced cyst recurrence after surgery and were repaired during the second surgery. Based on imaging findings, SEMCs mostly occurred in the thoracolumbar junction. Most of the fistulas (87.88%) were at the T12 or L1 levels. In patients with multiple adjacent SEMCs, the fistula may be at one end of the cyst rather than in the middle level of the cyst. A fistula laterality of 72.72% was consistent with cyst laterality. The JOA improvement rate was 61.84% ± 26.63%.
Conclusion Most fistulas were always located at the T12–L1 level as well as the middle level of the cyst, which is always consistent with cyst laterality. In patients with multiple adjacent SEMCs, the fistula may be at one end of the cyst. Cleft closure is key to healing SEMCs.
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Objective This study evaluated the feasibility and efficacy of quantitative reduction and fixation to treat basilar invagination (BI) with atlantoaxial dislocation (AAD).
Methods Posterior occipitocervical angle (POCA), occiput–C2 angle (Oc–C2A), clivusaxial angle (CAA), and C2–7 angle (C2–7A) were considered for quantitative reduction. Twelve patients with BI complicated with AAD received posterior interarticular release and individualized cage implantation to restore vertical dislocation. The POCA was adjusted using cantilever technology to further reduce the horizontal dislocation and adjust lower cervical vertebral angle. All patients received a radiological follow-up for ≥12 months. Improvements in spinal cord function were evaluated using Japanese Orthopedic Association (JOA) score.
Results All the patients received successful quantitative reduction for BI-AAD, and bony fusion was achieved without spinal cord injury after surgery for 12 months. The JOA score was improved significantly to 15.2 ± 0.9 twelve months after surgery (p < 0.01). Radiological follow-up revealed that individualized cage and POCA play vital roles in quantitative correction: (1) distance of the dens above McRae’s line and atlantodens interval were restored to normal level, respectively; (2) changes in Oc–C2 angle (ΔOc–C2A), C2–7 angle (ΔC2–7A), clivus-axial angle (ΔCAA), and POCA (ΔPOCA) were all caused by changes in axis tilt. Based on the changes of radiological parameter we deduced the formula for quantitative reduction by linear regression analysis: -ΔPOCA = ΔOc–C2A = -ΔC2–7A = ΔCAA.
Conclusion Quantitative posterior reduction by individualized cage and adjusting ΔPOCA is feasible for treating BI with AAD.
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