OBJECTIVE: To evaluate the effect of the basilar invagination (BI) type B on cervical spine.
METHODS: The research protocol used head magnetic resonance imaging (MRI) exams from 41 participants with BI type B and 158 controls. The criterion for BI was the distance of the odontoid apex to Chamberlain\'s line (DOCL) equal to or greater than 7 mm. The clivus length (CLI), clivus canal angle (CCA), Welcker\'s basal angle (WBA), Boogaard\'s angle (BOA), upper cervical lordosis angle (UCL), and total cervical lordosis angle (CL) were evaluated. The descriptive analysis, group comparisons, and correlations between skull base and cervical spine parameters were performed at the 95% confidence interval.
RESULTS: Participants with BI type B showed shorter clivus length (CLI: 25.7mm±7.3); greater angulation of the skull base (WBA: 126.5±10.4); greater inclination foramen magnum (BOA: 151.5±14.5); decrease in the value of the (CCA: 131.6±15); and greater angulations of UCL (17.9 ±13.8) and CL (29.7 ±19.9) in comparison to the control group (P < 0.05). Clivus length and CCA correlated inversely with UCL and CL, while BOA correlated directly with UCL and CL. The WBA did not correlate with CL (P < 0.05).
CONCLUSIONS: The deformation of skull base in the BI of type B caused, on average, a hyperlordosis of almost 30 degrees in the C2-C6 segment. This change was approximately 17º in the C2-C4, with the clivus hypoplasia being a risk factor for cervical hyperlordosis.

OBJECTIVE: The craniovertebral junction (CVJ) presents intricate anatomical challenges. In severe or irreducible malformations, complications such as reduction loss and fixation failure may occur, necessitating revision surgery. The posterior facet joint distraction and fusion (PFDF) technique, offers a solely posterior approach for revisions. Hence, we delineate varied revision scenarios, proposing surgical strategies and technical details to enhance outcomes and mitigate risks, thereby enriching the neurosurgical community\'s repertoire.
METHODS: This was a retrospective cohort study, analyzed patient data from Xuanwu Hospital, between 2017 to 2023. All patients had a history of surgical treatment for CVJ malformations, and experienced failure or loss of reduction. The distance from the odontoid process tip to the Chamberlain\'s line (DCL), the atlantodental interval (ADI), clivus-canal angle, cervicomedullary angle, width of subarachnoid space, CVJ area, and width of syrinx were used for radiographic assessment. Japanese Orthopaedic Association (JOA) scores and SF-12 scores were used for clinical assessment. Independent sample t-tests were employed. A significance level of p < 0.05 indicates statistically significant differences.
RESULTS: We analyzed data from 35 patients. For patients who underwent PFDF, the postoperative DCL, ADI, and clivus-canal angle significantly improved. For all patients, the postoperative cervicomedullary angle, width of subarachnoid space, CVJ area, and width of syrinx all demonstrated significant improvement, indicating the relief of neural compression. All patients showed significant improvement in both symptoms and clinical assessments.
CONCLUSIONS: Severe atlantoaxial joint locking or ligament contracting are the fundamental cause of reduction and fixation failure. Anterior odontoidectomy is indicated for patients with robust bony fusion of the atlantoaxial joint in an unreduced position. The PFDF technique is safe and effective for patients with incomplete atlantoaxial bony fusion. Preoperative assessment of surgical feasibility and vertebral artery status ensures surgical safety and efficacy.

UNASSIGNED: To describe the feasibility, safety and efficacy of mobilization of the vertebral artery for C2 pedicle screws in cases with the high-riding vertebral artery (HRVA).
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UNASSIGNED: During the period January 2020 to September 2022, fifteen patients underwent posterior occipitocervical fixation in our department. All patients had unilateral HRVA on at least one side that prohibited the insertion of C2 pedicle screws. There were 2 males and 13 females aged 47 ± 11.9 years (range: 17-64 years). After the correction of the vertical dislocation during the operation, the C2 pedicle screw insertion and occipitocervical fixation and fusion were performed using the vertebral artery mobilization technique. A routine three-dimensional reconstructed CT examination was executed to confirm the trajectory of C2 pedicle screws post-operation, and a CT angiography examination was performed when necessary. Neurological function was assessed using the Japanese Orthopedic Association (JOA) scale. The preoperative and postoperative JOA score and the main radiological measurements, including anterior atlantodental interval (ADI), the distance of odontoid tip above Chamberlain line, and clivus-canal angle (CCA), were collected and compared by paired t-test.
UNASSIGNED: All 15 patients had atlas assimilation, among which 12 patients had C2-C3 fusion (Klippel-Feil syndrome). Mobilization of the HRVA was successfully completed, and C2 pedicle screws were then fulfilled after the vertebral artery was protected. There was no injury to the vertebral artery during the operation. Meanwhile, no severe surgical complications such as cerebral infarction or aggravated neurological dysfunction occurred during the perioperative period. Satisfactory C2 pedicle screw placement and reduction were reached in all 15 patients. All the patients achieved bone fusion 6 months after surgery. No looseness and shift of internal fixation or reduction loss was observed during the follow-up period. Compared to the preoperative, the postoperative JOA score and the main radiological measurements were remarkably improved and statistically significant.
UNASSIGNED: C2 pedicle screw insertion assisted by mobilization of the vertebral artery is safe and considerably effective, providing a choice for internal fixation in cases with high-riding vertebral arteries.

OBJECTIVE: To investigate (1) lateral atlantoaxial articulation (LAA) morphology in patients with basilar invagination (BI) with atlantoaxial dislocation (AAD) and healthy individuals and its relationship with the severity of dislocation and (2) the effect of the LAA morphology on reduction degree (RD) after surgery.
METHODS: In this retrospective propensity score matching case-control study, imaging and baseline data of 62 patients with BI and AAD from 2011 to 2022 were collected. Six hundred thirteen  participants without occipitocervical junctional deformity served as controls. Logistic regression and receiver operating characteristic (ROC) curve were used for analysis.
RESULTS: The age, BMI and sex did not differ significantly between the two groups after propensity score matching. Sagittal slope angle (SSA) and coronal slope angle (CSA) was lower and greater, respectively, in the patient group than in the control group. A negative SSA value usually indicates anteverted LAA. Regression analysis revealed a significant negative correlation between SSA and severity of dislocation. However, no relationship was found between CSA and the severity of dislocation. The multivariate logistic regression analysis revealed that minimum-SSA emerged as an independent predictor of satisfactory reduction (RD ≥ 90%). The ROC curve demonstrated an area under the curve of 0.844, with a cut-off value set at -40.2.
CONCLUSIONS: SSA in patients group was significantly smaller and more asymmetric than that in the control group. Dislocation severity was related to SSA but not to CSA. Minimum-SSA can be used as a predictor of horizontal RD after surgery.

Klippel-Feil syndrome (KFS) is characterized by the congenital fusion of the cervical vertebrae and is sometimes accompanied by anomalies in the craniocervical junction. In basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the brainstem and cervical cord results in neurological defects and surgery is required. A 16-year-old boy diagnosed with KFS and severe BI presented with spastic tetraplegia, opisthotonus and dyspnea. CT scans showed basilar impression, occipitalization of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Posterior occipitocervical reduction and fusion along with decompression were performed. Paralysis gradually improved postoperatively over 3 weeks. However, severe spasticity and opisthotonus persisted and intrathecal baclofen (ITB) therapy was initiated. Following this, opisthotonus disappeared and spasticity of the extremities improved. Rehabilitation therapy continued by controlling the dose of ITB. Five years after the surgery, self-propelled wheelchair driving was achieved and activities of daily life improved. The treatment strategy for patients with BI and congenital anomalies remains controversial. Posterior reduction and internal fixation using instrumentation were effective techniques in this case. Spasticity control achieved through a combination of surgery and ITB treatment enabled the amelioration of therapeutic efficacy of rehabilitation and the improvement of ADL.

OBJECTIVE: Basilar impression (BI) is a rare yet debilitating abnormality of the craniovertebral junction, known to cause life-threatening medullary brainstem compression. Our study analyzes surgical approaches for BI and related outcomes.
METHODS: A systematic review was conducted using PubMed, Google Scholar, and Web of Science electronic databases according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to critically assess primary articles examining BI.
RESULTS: We analyzed 87 patients from 65 articles, mostly female (55.17%) with a mean age of 46.31 ± 17.94 years, commonly presenting with motor (59.77%) and sensory deficits (55.17%). Commonly employed procedures included posterior occipitocervical fusion (24.14%), anterior decompression (20.69%), and combined anterior decompression with posterior fusion (21.84%). Patients who underwent anterior approaches were found to be older (55.38 ± 17.67 vs. 45.49 ± 18.78 years, P < 0.05) and had a longer duration from symptom onset to surgery (57.39 ± 64.33 vs. 26.02 ± 29.60 months, P < 0.05) compared to posterior approaches. Our analysis revealed a significant association between a longer duration from symptom onset to surgery and an increased likelihood of undergoing odontoidectomy and decompression (odds ratio: 1.02, 95% confidence interval: 1.00-1.03, P < 0.05). Furthermore, after adjusting for all other covariates, a history of rheumatoid arthritis and the use of a posterior approach were significantly associated with an elevated risk of postoperative complications (P < 0.05).
CONCLUSIONS: The treatment approach to complex craniovertebral junction disease should be tailored to the surgeon\'s experience and the nature of the compressive pathology.

OBJECTIVE: We compared the diameter of the jugular foramen (JF) between normal individuals and those with a diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI).
METHODS: This cross-sectional analytical study measured the diameter of the right and left JF on magnetic resonance imaging scans of patients divided into 4 groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, and univariate analysis of variance with Tukey\'s post hoc test assessed JF differences between groups.
RESULTS: Considering all combinations of laterality and sex, the CMI+BI group had a JF narrower than their respective control groups. The JF was bilaterally narrower in the BI group than in the control group only in the women. Regardless of laterality, the CMI group had a wider JF than did the CMI+BI group in men and did not differ from that of the control group. In the female patients with CMI, the right JF did not differ from that of the controls and was wider than that in the CMI+BI group. Also, the left JF was narrower than that in the control group and did not differ from that in the CMI+BI group.
CONCLUSIONS: JF narrowing is associated with CMI and BI when combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claim a possible role of intracranial pressure disturbances as a triggering factor for CMI.

UNASSIGNED: Atlanto-occipital assimilation is the most common osseous anomaly of the cervical-occipital junction. The incidence rate of occipitalization has been reported 1.42.5/1000 children. Most of the patients are asymptomatic initially. Clinical features usually appear after 3rd decade depending on the level and degree of spinal cord compression with surrounding vital structures such as vertebral artery involvement. Hence, early definitive diagnosis and subsequent successful treatment are necessary in such cases to prevent life-threatening complications.
UNASSIGNED: A 16-year-old male presented with neck pain and progressive weakness of the bilateral lower limb since the past 2 months suffering from difficulty in walking, giddiness, and numbness of his fingertips. Hoffman\'s sign was positive. Plain cervical spine radiography (AP and lateral) is falsely suggestive of the absence of an atlas. MRI revealed the tip of the dens projecting 1314 mm above the Chamberlains line suggestive of basilar invagination. Occipito-cervical fixation was planned with an occipital plate, laminar, pedicle screw, and rods. Occipital plate fixed with screws (6,8 mm). C2 vertebra fixation with pedicle screw and laminar screws and C3 vertebra with a lateral mass screw under C-arm guidance. The patient mobilized with a walker from post-operative day 2 and then he ambulated unaided.
UNASSIGNED: Different types of atlanto-occipital assimilation have been reported. Treatment modality completely depends on neurological symptoms and instability.Although, different surgical options are available, occipito-cervical fixation with occipital plate, screws, and rod fixation is the most superior surgical technique for atlanto-occipital assimilation with atlanto-occipital instability.

Objectives and Background: To present a novel technique of treatment for a patient with basilar invagination. Basilar invagination (BI) is a congenital condition that can compress the cervicomedullary junction, leading to neurological deficits. Severe cases require surgical intervention, but there is debate over the choice of approach. The anterior approach allows direct decompression but carries high complication rates, while the posterior approach provides indirect decompression and offers good stability with fewer complications. Materials and Methods: A 15-year-old boy with severe myelopathy presented to our hospital with neck pain, bilateral upper limb muscle weakness, and hand numbness persisting for 4 years. Additionally, he experienced increased numbness and gait disturbance three months before his visit. On examination, he exhibited hyperreflexia in both upper and lower limbs, muscle weakness in the bilateral upper limbs (MMT 4), bilateral hypoesthesia below the elbow and in both legs, mild urinary and bowel incontinence, and a spastic gait. Radiographs revealed severe basilar invagination (BI). Preoperative images showed severe BI and that the spinal cord was severely compressed with odontoid process. Results: The patient underwent posterior surgery with the C-arm free technique. All screws including occipital screws were inserted into the adequate position under navigation guidance. Reduction was achieved with skull rotation and distraction. A follow-up at one year showed the following results: Manual muscle testing results and sensory function tests showed almost full recovery, with bilateral arm recovery (MMT 5) and smooth walking. The cervical Japanese Orthopedic Association score of the patient improved from 9/17 to 16/17. Postoperative images showed excellent spinal cord decompression, and no major or severe complications had occurred. Conclusions: Basilar invagination alongside Klippel-Feil syndrome represents a relatively uncommon condition. Utilizing a posterior approach for treating reducible BI with a C-arm-free technique proved to be a safe method in addressing severe myelopathy. This novel navigation technique yields excellent outcomes for patients with BI.