Biomechanical differences of three cephalic fixation methods for patients with basilar invagination and atlantoaxial dislocation in the setting of congenital atlas occipitalization: a finite element analysis.

BACKGROUND CONTEXT: In cases of basilar invagination-atlantoaxial dislocation (BI-AAD) complicated by atlas occipitalization (AOZ), the approach to cranial end fixation has consistently sparked debate, generally falling into two categories: C1-C2 fixation and occipitocervical fixation. Several authors believe that C1-C2 fixation carries a lower risk of fixation failure than occipitocervical fixation. PURPOSE: To study the biomechanical differences among 3 different cranial end fixation methods for BI-AAD with AOZ. STUDY DESIGN: This was a finite element analysis. PATIENT SAMPLE: A 35-year-old female patient diagnosed with congenital BI-AAD and AOZ. OUTCOME MEASURES: range of motion (ROM), peak von Mise stress (PVMS), cage micro-subsidence, cage micro-slippage METHOD: Four finite element models were constructed, including unstable group (BI-AAD with AOZ), C1 lateral mass screw group, occipital plate group, occipitocervical rod group. The flexion and extension (FE), lateral bending (LB) as well as axial rotation (AR) were simulated under a torque of 1.5 Nm. Parameters include C1-C2 ROM, PVMS on screw-rod construct, cage micro-subsidence, cage micro-slippage. RESULTS: The ROM of the C1 lateral mass screw group was smaller than that of the other fixation groups in LB and AR, but not FE. Compared with the occipitocervical rod group, the ROM in LB and AR of the occipital plate group was higher, but not in FE. The PVMS of C1 lateral mass screw group was significantly higher than that of the other groups. The ROM and PVMS of the occipitocervical rod group were in between the other 2 groups. Regarding the screws at the cranial end, the PVMS of the 4-screw occipitocervical rod group was significantly lower than that of the other groups. In general, the cage micro-motion follows the ascending order: C1 lateral mass group < occipitocervical rod group < occipital plate group. CONCLUSION: In cases of BI-AAD with AOZ, the C1 lateral mass screw group provided the least ROM and cage micro-motion, but the screw-rod PVMS was the largest. The advantage of occipital plate fixation lies in the lowest screw-rod PVMS, but the ROM and cage micro-motion is the highest. Four-screw fixation at the cranial end of occipitocervical rod group helps to reduce the PVMS and may prevent screw failure at the cranial end.

A screw algorithm for congenital C2-3 fusion with high-riding vertebral arteries: feasibilities and clinical outcomes of five different fixation techniques.

OBJECTIVE: To propose a screw algorithm and investigate the anatomical feasibilities and clinical outcomes of five distinct fixation methods for C2-3 fused vertebra with high-ridding vertebral arteries (VA) (HRVA) when the C2 pedicle screw placement is unfeasible. METHODS: Thirty surgical patients with congenital C2-3 fusion, HRVA, and atlantoaxial dislocation (AAD) were included. We designed a algorithm for alternative screw implantation into C2-3 fused vertebrae, including C2 pedicle screw with in-out-in (passing VA groove) technique (in-out-in screw), subfacetal screw, translaminar screw, lateral mass screw, C3 pedicle screw. VA diameter and position, C2 and C3 pedicles, superior facets, fused lamina, and fused lateral mass dimensions were evaluated for screw implantation indication. Implant failure, reduction loss, implant placement accuracy were investigated by computed tomography. RESULTS: A total of 5 VAs were identified as distant VAs; a total of 2 VAs were categorized as occlusive VAs. Sufficient dimension of lateral mass and lamina provided the broadest indications for screw implantation, while the distant or occlusive VA provided the most limited indications for in-out-in screw. The indications of five alternative methods ranged from narrowest to widest as follows: in-out-in screw, C3 pedicle screw, subfacetal screw, translaminar screw, lateral mass screw. The translaminar screws and the lateral mass screws increased the probability of implant failure. All patients who received in-out-in screws, C3 pedicle screws, and subfacetal screws achieved fusion. The accuracy ranged from lowest to highest as follows: C3 pedicle screw, lateral mass screw, in-out-in screw, subfacetal screw, translaminar screw. No translaminar screws deviated. CONCLUSIONS: The algorithm proved to be a valuable tool for screw selection in cases of C2-3 fused vertebrae with HRVAs. The subfacetal screw, boasting broad indications, a high fusion rate, and exceptional accuracy, stood as the primary preferred alternative.

Modified interfacet technique using shaped autologous occipital bone mass for basilar invagination.

PURPOSE: In economically undeveloped areas, surgery for basilar invagination (BI) is still a serious economic burden for people. This study introduces a modified interfacet technique for the treatment of BI using shaped autologous occipital bone mass to reduce BI and to save economical expenditure. METHODS: The data of 6 patients with BI who underwent modified interfacet technique using shaped autologous occipital bone mass in our hospital from April 2020 to February 2021 were retrospectively analyzed. During the operation, osteotomy at the external occipital protuberance was performed using ultrasonic osteotome, followed by interfacet release and implantation of shaped autologous occipital bone mass to complete vertical reduction. The atlantodental interval (ADI), Chamberlain's line violation (CLV), clivo-axial angle (CXA) and cervico-medullary angle (CMA) were compared before and after surgery. Additionally, we observed implant stability during the follow-up period to assess the long-term success of the modified interfacet technique. RESULTS: The surgical procedure was successful in all six patients, with no reported incidents of vascular injury, spinal cord injury, or dural tear. Following the operation, improvements were observed in the ADI, CLV, CXA, and CMA. Throughout the follow-up period, the implants remained stable, demonstrating no complications such as bone resorption of the autologous occipital bone mass, implant fracture, or displacement. CONCLUSION: The utilization of shaped autologous occipital bone mass in atlantoaxial interfacet bone grafting has demonstrated effectiveness and feasibility. This technique offers simplicity, ease of preparation, and cost-effectiveness, making it a viable option for treating BI.

Clinical Characteristics and Treatment Outcomes of Long-Level Intramedullary Spinal Cord Tumors: A Consecutive Series of 43 Cases.

OBJECTIVE: Long-level intramedullary spinal cord tumors (LIMSCTs) cause complex treatment issues. However, LIMSCTs have rarely been analyzed separately. The authors reported a large case series of LIMSCTs and analyzed the clinical characteristics and treatment outcomes. METHODS: The medical data of patients with LIMSCTs at our institution between January 2015 and December 2019 were retrospectively reviewed. Demographics, tumor size and location, pathology, extent of resection, and neurological functional status were collected. RESULTS: A total of 43 consecutive cases were included. Twenty-three cases (53.5%) of LIMSCTs were ependymal tumors. All patients with ependymal tumors achieved gross total resection (GTR). In ependymal tumor cases, 3 cases (13%) of ependymal tumors experienced postoperative neurological deterioration, and 66% of them showed an improvement at follow-up; 25.6% were low-grade astrocytic tumors. The rates of GTR, subtotal resection (STR) and partial resection (PR) were 63.6%, 27.3%, and 9.1%, respectively. Twenty-seven percent cases showed postoperative neurological worsening, and 33% of them had an improvement at follow-up; 20.9% were high-grade astrocytic tumors. The excision rates were 44.4% for GTR, 44.4% for STR, and 11% for PR, respectively. Fifty-five percent cases showed postoperative neurological worsening, and none of them had an improvement at follow-up. CONCLUSION: In this series, all LIMSCTs were gliomas. Aggressive tumor resection did not increase the risk of long-term functional deterioration in ependymal tumors and low-grade astrocytic tumors, but in high-grade astrocytic tumors, patients had a higher risk of neurological deterioration and difficulty in recovery. In ependymal tumors and low-grade astrocytic tumors, patients can achieve long-time survival after performing aggressive tumor resection.

Does H3K27M Mutation Impact Survival Outcome of High-Grade Spinal Cord Astrocytoma?

OBJECTIVE: To evaluate the impact of H3K27M mutation in the prognosis of histological high-grade intramedullary astrocytoma. METHODS: A total of 78 patients who were diagnosed with high-grade spinal cord astrocytoma were included. Clinical data consisting demographic, radiological, molecular features and treatment data were recorded. Univariate and multivariate Cox analysis were performed to investigate variables associated with survival outcome of histological high-grade spinal cord astrocytoma. RESULTS: Median survival time was 21 months. Overall survival (OS) at 1 and 3 years was 65.7% and 40.7%, respectively. Sex, location, and tumor span did not present significant association with OS. Patients with H3K27M mutation showed significant shorter duration of symptom than patients with H3K27 wild-type. As respect to adjuvant treatment, adjuvant radiotherapy and chemotherapy were associated with favorable OS (both p = 0.01). Younger patients (age ≤ 18 years) had shorter OS (p = 0.008) than adult patients (age > 18 years). Of note, H3K27M mutation did not show significant impact on the survival outcome, regardless of histology grade 3 or grade 4 (p = 0.3). CONCLUSION: Histological high-grade spinal cord astrocytoma has dismal prognosis. Our study demonstrated that H3K27M mutation did not show significant impact on survival outcome of histological high-grade spinal cord astrocytoma.

Basilar invagination without atlantoaxial dislocation: treatment by correction of clivus canal angle with interfacet distraction and fixation.

BACKGROUND: This study reports on the surgical technique used and clinical outcomes obtained during the treatment of basilar invagination (BI) without atlantoaxial dislocation (AAD) through the correction of the clivus canal angle (CCA) using interfacet distraction and fixation. METHODS: Nineteen cases with BI without AAD treated by the correction of the clivus canal angle were retrospectively analyzed. Pre- and postoperative computed tomography scans and three-dimensional reconstruction views were obtained to measure the size of the CCA, pB-C2 distance, and degree of BI. Chiari malformation and syringomyelia were evaluated by magnetic resonance imaging (MRI). The clinical outcomes for all patients were measured using the Japanese Orthopedic Association (JOA) scale. The CCA was corrected by using interfacet distraction and fixation techniques. The Wilcoxon test was used to compare pre- and postoperative measurements. RESULTS: All the patients were followed up for 24.95 ± 5.22 months (range 12-36 months); no patient suffered intraoperative nerve or vascular injury. Clinical symptoms improved in 17 patients (89.5%). The mean JOA score increased from 12.32 ± 1.89 to 14.37 ± 1.30 (Z = -3.655, P < 0.001). The mean CCA improved from 129.34 ± 8.52° preoperatively to 139.75 ± 8.86° postoperatively (Z = -3.824, P < 0.001). The mean pB-C2 decreased from 7.47 ± 2.21 to 5.68 ± 3.13 (Z = -3.060, P = 0.002). Syringomyelia was significantly reduced in 10 out of 13 patients by the first follow-up year. All patients achieved bony fusion. CONCLUSION: Posterior interfacet distraction and fixation to correct the CCA is a feasible and effective method for treating BI without AAD.

Application of C2 subfacetal screws for the management of atlantoaxial dislocation in patients with Klippel-Feil syndrome characterized by a narrow C2 pedicle and high-riding vertebral artery.

OBJECTIVE: This study aims to investigate the clinical application and feasibility of C2 subfacetal screws in patients with Klippel-Feil syndrome (KFS), narrow C2 pedicles, and high-riding vertebral arteries (HRVAs). METHODS: The clinical data of seven patients with KFS, atlantoaxial dislocation, narrow C2 pedicles, and HRVAs treated with C2 subfacetal screws were analyzed in this retrospective study. The internal height, isthmus height, and pedicle width of C2 vertebra were measured using preoperative computed tomography (CT). Subfacetal screws were inserted for 7 patients (12 sides). The position and length of the screws were observed using postoperative CT. Intraoperative dura mater and vertebral artery (VA) injuries were recorded. Bone fusion was observed using follow-up CT. RESULTS: The internal height was 10.5 ± 3.2 mm, the isthmus height was 3.7 ± 1.8 mm, the pedicle width was 3.0 ± 1.4 mm, and the screw length was 19.7 ± 1.5 mm. All patients had HRVAs and narrow pedicles. No injury to the dura mater and vertebral artery (VA) occurred in this group of patients. Bone fusion was achieved in all patients during follow-up. CONCLUSIONS: In patients with KFS, HRVA, and a narrow C2 pedicle, there is sufficient space below the C2 articular surface for screw insertion. When the pedicle is narrow and the C2 pedicle screw is not suitable for placement due to possible injury to the VA, subfacetal screws are a feasible alternative.

Spinal intradural schwannoma presenting with acute subarachnoid hemorrhage: a case report and review of published reports.

Acute subarachnoid hemorrhage (SAH) presentation is a very rare occurrence in spinal schwannomas. We report a case of lumbar schwannoma in a patient presenting first with acute SAH. A 40-year-old man was referred to our department 7 days after the sudden onset of headache, neck pain, and fever. SAH was suspected; however, head computed tomography (CT) findings were normal. A lumbar puncture indicated blood-stained cerebrospinal fluid (CSF). Moreover, the CSF pressure changed from 200 mmH2O to 90 mmH2O after drainage of 10 mL of CSF indicating a blockage of CSF. Subsequent magnetic resonance imaging (MRI) confirmed an intradural tumor with SAH, which also caused blockage of the CSF circulation. The patient underwent immediate surgery and fully recovered. In conclusion, the early diagnosis and total removal of the tumor and blood clot significantly improved the patient's outcome. There is a high index of suspicion for spinal tumors resulting in SAH when there is a CSF pressure change after lumbar puncture in an SAH patient.

Microarray analysis of aberrant microRNA expression patterns in spinal cord gliomas of different grades.

MicroRNAs (miRNAs) are involved in the development of several types of tumor; however, their role in spinal gliomas remains unknown. The present study aimed to identify potentially novel spinal cord gliomas (SCG)-associated miRNAs and to characterize their roles in the development and progression of SCG. miRNA expression levels in low-grade SCG (classed as stage I-II SCG based on the World Health Organization grading system), high-grade SCG (classed as stage IV SCG based on the World Health Organization grading system) and 5 control cases were measured using a miRNA expression microarray. Subsequently, blood samples from the spinal cord of patients with differing grades of SCG were screened for differentially expressed miRNAs (DEmiRNAs). Compared with the control group, 7 upregulated and 36 downregulated miRNAs were identified in the low-grade SCG group and a total of 70 upregulated and 20 downregulated miRNAs were identified in the high-grade SCG group (P≤0.05, fold change >2). Gene Ontology analysis revealed that the regulation of cellular metabolic processes, negative regulation of biological processes and axon guidance were primarily involved. Moreover, pathway analysis showed that the target genes of DEmiRNAs were enriched in tumor-related signaling pathways, such as the MAPK and Wnt signaling pathway. The results suggest that DEmiRNAs in peripheral blood may serve as novel target markers with high specificity and sensitivity for the diagnosis of SCG.

Publisher Correction: Comparison of Alterations in miRNA Expression in Matched Tissue and Blood Samples during Spinal Cord Glioma Progression.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Comparison of Alterations in miRNA Expression in Matched Tissue and Blood Samples during Spinal Cord Glioma Progression.

Abnormal expression of microRNAs (miRNAs) contributes to glioma initiation. However, the expression of miRNAs in tumour tissue or blood of spinal cord glioma (SCG) patients, particularly in high-grade spinal gliomas (Grade IV) known as glioblastoma (GBM), remains largely unknown. In this study we aimed to determine differentially expressed miRNAs (DEmiRNAs) in the tissue and blood between spinal cord glioblastoma (SC-GBM) patients and low grade SCG (L-SCG) patients. Additionally, we predicted key miRNA targets and pathways that may be critical in glioma development using pathway and gene ontology analysis. A total of 74 miRNAs were determined to be differentially expressed (25 upregulated and 49 downregulated) in blood, while 207 miRNAs (20 up-regulated and 187 down-regulated) were identified in tissue samples. Gene ontology analysis revealed multicellular organism development and positive regulation of macromolecule metabolic process to be primarily involved. Pathway analysis revealed "Glioma", "Signalling pathways regulating pluripotency of stem cells" to be the most relevant pathways. miRNA-mRNA analysis revealed that hsa-miRNA3196, hsa-miR-27a-3p, and hsa-miR-3664-3p and their target genes are involved in cancer progression. Our study provides a molecular basis for SCG pathological grading based on differential miRNA expression.

A modified microsurgical interfacet release and direct distraction technique for management of congenital atlantoaxial dislocation: technical note.

Various techniques have been used for management of congenital atlantoaxial dislocation. Recently, the reduction of atlantoaxial dislocation through a single posterior approach has attracted more and more attention. Here, we present a modified technique including direct interfacet release and distraction between C1 and C2 by a specially designed distractor, posterior internal fixation and bone graft fusion. The illustrated technique was performed in 15 consecutive patients, and the outcomes were recorded and analyzed. Follow-up ranged from 12 to 26 months. Clinical symptoms improved in 14 patients (93.3%) and were stable in 1 patient (6.7%). Radiologically, 60-100% reduction was achieved in 13 patients (86.6%). Bone fusion was obtained in all patients at 12 months after the operation. The two-tailed Wilcoxon signed-rank test was used to analyze the preoperative and postoperative Japanese Orthopedic Association scores (JOA), atlas-dens interval (ADI), and cervicomedullary angle (CMA) (P < 0.001). Our results suggested that this direct interfacet release and distraction technique with a specially designed C1-2 distractor can provide a definite effective C1-2 facet distraction and odontoid process restoration through a single posterior approach.

Surgical management of Chiari I malformation based on different cerebrospinal fluid flow patterns at the cranial-vertebral junction.

Chiari I malformation has been shown to present different cerebrospinal fluid (CSF) flow patterns at the cranial-vertebral junction (CVJ). Posterior fossa decompression is the first-line treatment for symptomatic Chiari I malformation. However, there is still controversy on the indication and selection of decompression procedures. This research aims to investigate the clinical indications, outcomes, and complications of the decompression procedures as alternative treatments for Chiari I malformation, based on the different CSF flow patterns at the cranial-vertebral junction. In this study, 126 Chiari I malformation patients treated with the two decompression procedures were analyzed. According to the preoperative findings obtained by using cine phase-contrast MRI (cine PC-MRI), the abnormal CSF flow dynamics at the CVJ in Chiari I malformation was classified into three patterns. After a preoperative evaluation and an intraoperative ultrasound after craniectomy, the two procedures were alternatively selected to treat the Chiari I malformation. The indication and selection of the two surgical procedures, as well as their outcomes and complications, are reported in detail in this work. Forty-eight patients underwent subdural decompression (SDD), and 78 received subarachnoid manipulation (SAM). Ninety patients were diagnosed as having Chiari I malformation with a syrinx. Two weeks after the operation, the modified Japanese Orthopedic Association (mJOA) scores increased from the preoperative value of 10.67 ± 1.61 to 12.74 ± 2.01 (P < 0.01). The mean duration of follow-up was 24.8 months; the mJOA scores increased from the postoperative value of 12.74 ± 2.01 to 12.79 ± 1.91 at the end of follow-up (P = 0.48). More complications occurred in the patients who underwent SAM than in those who received SDD (SAM 11 of 78 (9.5%) vs SDD 2 of 48 (3.5%)). The abnormal CSF flow dynamics at the CVJ in Chiari I malformation can be classified into three patterns. A SAM procedure is more feasible in Chiari I malformation (CM1) patients with pattern III CSF flow dynamics, whereas a SDD procedure is more suitable for CM1 patients with pattern I CSF flow dynamics. In CM1 patients with pattern II CSF flow dynamics, an intraoperative ultrasound after craniectomy could play an important role in the selection of an effective decompression procedure.

Spinal cord gliosarcoma with rhabdomyoblastic differentiation: a case report.

Very few cases of gliosarcoma (GS) in the spinal cord with or without rhabdomyoblastic differentiation have been reported at young ages, leading to limited information on the clinical, pathological and prognosis of this type of tumors. We report a case of GS with rhabdomyoblastic differentiation in a 6-year-old girl in C1-C6 level spinal cord. This is, to the best of our knowledge, the first report of GS with rhabdomyoblastic differentiation primarily developed in spinal cord at such a young age. Histologically, GS is composed of both glioblastoma components and malignant mesenchymal components. In the present case, the mesenchymal portion displayed a typical pattern of rhabdoid morphology. The rhabdomyoblastic-differentiated cells were confirmed by desmin, MyoD1, myogenin and Vimentin immunopositivity. Loss of PTEN (phosphatase and tensin homolog) and amplification of EGFR (epidermal growth factor receptor) were not detected in both parts of GS (glioblastoma component and rhabdomyosarcoma component). Interestingly, in this case rhabdomyoblastic-differentiated cells (rhabdomyosarcoma component) were focally negative for integrase interaction 1 (INI-1) protein and glial cells (glioblastoma component) were positive, and monosomy 22 in the former and absence in the latter. The patient only received low-dose radiotherapy and survived only 6 months after diagnosis. GSs with rhabdomyoblastic differentiation have a worse prognosis than common GSs and high-dose radiotherapy is suggested to considerer.

Treatment of selected syringomyelias with syringo-pleural shunt: the experience with a consecutive 26 cases.

OF BACKGROUND DATA: It is well established that syringomyelia can cause neurological symptoms and deficit by accumulation of fluid within syrinx cavities that lead to internal compression within the spinal cord. When other intervention treating the underlying etiology failed to yield any improvement, the next option would be a procedure to divert the fluid from the syrinx cavity, such as syringo-subarachnoid, syringo-peritoneal or syringo-pleural shunting. The indications and long term efficacy of these direct shunting procedures are still questionable and controversial. OBJECTIVE: To investigate the clinical indication, outcome and complication of syringe-pleural shunt (SPS) as an alternative for treatment of syringomyelia. STUDY DESIGN: We reported a retrospective 26 cases of syringomyelia were found to have indication for a diversion procedure. SPS was offered. Patients' symptoms, mJOA score, and MRI were collected to evaluate the change of the syringomyelia and prognosis of the patients. 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. METHODS: All 26 patients underwent SPS. The clinical information was collected, the mean follow-up time was 27.4 months, 2-tailed wilcoxon signed-rank test was used to perform the statistical analysis of the mJOA scores. The key surgical technique, outcome and complications of SPS were reported in detail. RESULTS: No mortality and severe complications occurred. Postoperative MRIs revealed near-complete resolution of syrinx in 14 patients, significant shrinkage of syrinx in 10 patients, no obvious reduction or unchanged in remaining 2 patient. Postoperatively, the symptoms improved in 24 cases (92.3%). Statistical analysis of the mJOA scores showed a statistical significance (P<0.001) between the preoperative group and the 2-week postoperative group. No further significant improvement between 2 weeks to the final follow up at 27 months. CONCLUSION: Collapse or remarkable shrinkage of the syrinx by SPS could ameliorate or at least stabilize the symptoms for the patient. We recommend small laminectomy and a less than 3mm myelotomy either at PML or DREZ. The SPS procedure can be an effective and relatively long-lived treatment for the idiopathic syringomyelia and those that failed other options.

[Application of intraoperative contrast-enhanced ultrasound in different pathological grades of glioma].

OBJECTIVE: To explore the feasibility and value of intra-operative contrast-enhanced ultrasound in different pathological grades of glioma. METHODS: A total of 46 patients with glioma were selected to undergo the contrast-enhanced ultrasound. They were divided into 3 groups according to their pathological grades. RESULTS: All 46 tumors were hyperechoic after examinations and the borders became much more distinct. Different level of tumors had their representative ultrasonic findings. CONCLUSIONS: Intraoperative contrast-enhanced ultrasound is quite useful for determining the position and border of tumors. And it is also excellent in detecting the residual tumor tissues post-operatively. And it can improve the resection accuracy and operative precision, especially for Grade IV glioma with internal necrosis, cyst and surrounding brain edema.