Sagittal slope angle of lateral atlantoaxial articulation is associated with the severity of basilar invagination with atlantoaxial dislocation and predicts reduction degree after surgery.

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. CONCLUSION: 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.

Are Chiari Malformation and Basilar Invagination Associated with Jugular Foramen Stenosis?

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.

Correlation Among Syrinx Resolution, Cervical Sagittal Realignment, and Surgical Outcome After Posterior Reduction for Basilar Invagination, Atlantoaxial Dislocation, and Syringomyelia.

BACKGROUND: The correlation among syrinx resolution, occipitoaxial sagittal alignment, and surgical outcome in long-term follow-up seems to have not been clarified. OBJECTIVE: To further explore the relationship between the syrinx resolution and occipitoaxial realignment after posterior reduction and fixation in basilar invagination (BI)-atlantoaxial dislocation (AAD) patients with syringomyelia. METHODS: A continuous series of 32 patients with BI-AAD and syringomyelia who received direct posterior reduction met the inclusion criteria of this study. Their clinical and imaging data were analyzed retrospectively. Before surgery and at the last follow-up, we used the Japanese Orthopedic Association (JOA) score and the Neck Disability Index (NDI) to assess the neurological status, respectively. The Pearson correlation coefficient and multiple stepwise regression analysis were used to explore the relevant factors that may affect surgical outcomes. RESULTS: There were significant differences in atlantodental interval, clivus-axial angle, occiput-C2 angle (Oc-C2A), cervicomedullary angle (CMA), subarachnoid space (SAS) at the foramen magnum (FM), syrinx size, NDI, and JOA score after surgery compared with those before surgery. ΔCMA and the resolution rate of syrinx/cord as relevant factors were correlated with the recovery rate of JOA (R 2 = 0.578, P < .001) and NDI (R 2 = 0.369, P < .01). What's more, ΔSAS/FMD (SAS/FM diameter) and ΔOc-C2A were positively correlated with the resolution rate of syrinx/cord (R 2 = 0.643, P < .001). CONCLUSION: With medulla decompression and occipital-cervical sagittal realignment after posterior reduction and fusion for BI-AAD patients with syringomyelia, the structural remodeling of the craniovertebral junction and occipitoaxial realignment could contribute to syringomyelia resolution.

C1-2 Fusion in Atlantoaxial Dislocation and Basilar Invagination with and without Chiari Malformation: Clinical/Radiological and Craniometric Results.

OBJECTIVE: Patients with Chiari malformation (CM) associated with atlantoaxial dislocation (AAD) and basilar invagination (BI) may present with a small posterior cranial fossa, but data on the volumetric analysis are lacking. Additionally, whether additional foramen magnum decompression (FMD) is needed together with atlantoaxial fusion remains controversial. This study evaluated the volumetric alterations of the posterior cranial fossa in these patients and analyzed the radiological and clinical outcomes after posterior C1-C2 reduction and fixation plus C1 posterior arch resection. METHODS: Thirty-two adult CM patients with AAD and BI (CM-AAD/BI group) and 21 AAD and BI patients without CM (AAD/BI-only group) who received posterior atlantoaxial fusion plus C1 posterior arch resection were retrospectively studied. The clinical and radiological outcomes and volumetric measurements of the posterior cranial fossa were evaluated. RESULTS: The majority of CM-AAD/BI patients (94%) improved clinically and radiologically at 12 mo postoperatively, and none required additional FMD. Morphological analysis revealed a significant reduction in the bony posterior cranial fossa volumes of the CM-AAD/BI group (P < 0.01) and the AAD/BI-only group (P < 0.01) relative to those of the CM group. No significant differences were observed between the CM-AAD/BI and AAD/BI groups. CONCLUSIONS: Compared with patients with simple CM, patients with AAD/BI with or without CM demonstrated a considerably and equally reduced bony posterior cranial fossa volume. No additional FMD is needed in the treatment of CM-AAD/BI patients after posterior reduction and fusion plus C1 posterior arch resection.

Chamberlain's Line Violation in Basilar Invagination Patients Compared with Normal Subjects: A Systematic Literature Review and Meta-Analysis.

OBJECTIVE: To compare the measured odontoid tip violation above Chamberlain's line described in the literature to diagnose basilar invagination (BI) and to establish the normal placement of the dens tip defining individuals without BI (normal subjects). METHODS: A systematic literature review was performed to identify clinical or radiological studies that expressed the amount of odontoid violation above Chamberlain's line in patients with a BI diagnosis. In addition, a meta-analysis was performed to evaluate normal subjects' values of Chamberlain's line violation (CLV). RESULTS: There were 23 studies included (13 radiological and 10 clinical). Most studies used computed tomography and/or magnetic resonance imaging. Eight different cutoff values were used to measure dislocated odontoid apexes above Chamberlain's line regardless of the radiological modality. The mean measured amount of CLV was 3.95 mm (median 5 mm; range, 0-9 mm). The meta-analysis included 8 studies (1233 patients) with a normal sample population with a mean normal CLV of -0.63 mm (below the line) (95% confidence interval [-0.8, 1.18 mm], random effects model). CONCLUSIONS: Different values were found in the assessed studies used for CLV in BI diagnosis. This variability is especially important for type B BI, as type A BI has other craniocervical diagnostic parameters. Considering the results obtained in this meta-analysis, BI should be diagnosed in the case of any dens violation >1.18 mm.

Association between Brachycephaly, Chiari Malformation, and Basilar Invagination.

BACKGROUND: There is evidence that Chiari malformation (CM) and basilar invagination (BI) are largely due to disproportion between the content and volume of the posterior fossa. A recent study identified an increased association between brachycephaly and BI. In several types of craniosynostosis, the posterior fossa volume is smaller than normal, and this is more pronounced in coronal synostosis. The aim of this study is to evaluate the association between CM and BI. METHODS: The cephalic index (CI) measured on magnetic resonance imaging (MRI) from a sample of patients with craniocervical malformation was compared with that of normal subjects. RESULTS: The average CI in the craniovertebral junction malformation (CVJM) group was significantly higher in BI patients than in normal subjects. The BI patients also had the highest CI among the whole sample of patients (p = 0.009). CONCLUSIONS: In this study, BI patients had the highest CI among patients with CVJM and a significantly higher CI than those in the control group. Our data confirm the association between BI and brachycephaly.

Are plain radiographic measurements still consistent with a diagnosis of basilar invagination in the era of cross-sectional images?

Retrospective cross-sectional study To evaluate the validity and obtain optimal cutoff values of 3 radiologic measurements for the diagnosis of basilar invagination (BI). Two hundred seventy-six patients (46 patients who underwent atlantoaxial fusion for BI and 230 patients who were treated for minor cervical trauma) seen in a single institution from January 2010 to December 2016 were included in this study. Age, sex, and body mass index were adjusted for the patients. The Ranawat index (RI), modified Ranawat method (MRM), and Redlund-Johnell method (RJM) were used to diagnose BI on plain radiographs. The sensitivity, specificity, positive predictive value, negative predictive value, accuracy, and diagnostic odds ratio of 3 radiologic measurements were compared. We also calculated the optimized cutoff values of 3 radiologic measurements using the receiver operating characteristic curve in our patients. The mean age of the 130 women and 146 men was 58.3 ±â€…14.5 years. The mean values of RI, MRM, and RJM in the BI group were 12.5 ±â€…3.3, 23.1 ±â€…3.8, and 27.3 ±â€…3.6 in women and 13.6 ±â€…2.6, 26.8 ±â€…4.2, and 34.7 ±â€…5.1 in men. There was a significant difference between the sexes (P < .05). The accuracies of RI, MRM, and RJM were 95%, 89.6%, and 92.3% in women and 93%, 68.2%, and 85.4% in men, respectively. The optimized cutoff values of RI, MRM, and RJM were 14, 26, and 32 mm in women and 15, 29, and 38 mm in men. Three radiologic measurements (RI, MRM, and RJM) are reliable for the diagnosis of BI even in the era of cross-sectional images. The validity of these measurements depends on sex and particular radiologic measurement. The optimized cutoff values of RI, MRM, and RJM were 14, 26, and 32 mm in women and 15, 29, and 37 mm in men. These cutoff values showed high validity when compared to the CT and MRI findings.

Investigating Type B Basilar Invagination Through Cephalic Indices.

OBJECTIVE: We sought to compare the diagnostic accuracy of cephalic indices for type B basilar invagination (BI). METHODS: This retrospective study examined head and neck magnetic resonance imaging sequences of 31 Group B BI cases and 96 controls. Two examiners blinded to diagnostic data evaluated the cephalic indices of each magnetic resonance imaging sequence, described as width/length (WLI) and height/width (HWI). The distance of the odontoid process apex to Chamberlain line and clivus canal angle were measured. The interexaminer and intraexaminer reproducibility of the cephalic indices was calculated using intraclass correlation coefficient. The diagnostic accuracy was discerned by the receiver operating characteristic (ROC) curve. All analyses were scrutinized with a 95% confidence interval. RESULTS: Cephalic indices showed interexaminer and intraexaminer reproducibility ≥94%. The areas under the ROC curve were 0.639 (WLI) and 0.874 (HWI) (95% confidence interval: P < 0.05). The HWI showed a sensitivity of 74.7% and a specificity of 85.5% for the cutoff criterion ≤58. The WLI presented a sensitivity of 53.3% and a specificity of 66.7% for the cutoff criterion ≥86. CONCLUSIONS: The HWI showed the largest area under the ROC curve in comparison with the WLI, with robust sensitivity and specificity values, indicating that the proportions between cranial height and width can help clinicians in investigating type B BI.

Evaluating Craniovertebral Stability in Chiari Malformation Coexisting with Type II Basilar Invagination: An Observational Study Based on Kinematic Computed Tomography and Its Clinical Application.

BACKGROUND: Treatment of Chiari malformation (CM) is controversial, especially when it coexists with "stable" or Type II basilar invagination (CM + II-BI). Precise evaluation of craniovertebral junction (CVJ) stability is crucial in such patients; however, this has never been validated. This study aimed to dynamically evaluate atlanto-condyle and atlantoaxial stability by kinematic computed tomography (CT) and report its surgical treatment. METHODS: The study recruited 101 patients (control, CM, and CM + II-BI groups: 48, 34, and 19 patients, respectively). During kinematic CT, the CVJ stability-related parameters were measured and compared between the 3 groups. The surgical strategy for treating CM + II-BI was based on these results. Preoperative and postoperative images were acquired, and functional scores were used to assess the outcome. RESULTS: Among the 3 groups, the length of the clivus and the height of the condyle were the shortest in the CM + II-BI group, which was accompanied by the greatest rotation of the atlas and atlanto-condyle facet movement on cervical flexion and extension. Moreover, in such patients, increased Chamberlain's baseline violation indicated the aggregate invagination of the odontoid in the flexed position, and asymmetric displacement of atlantoaxial facets was observed. Seventeen CM + II-BI patients underwent surgical treatment with atlantoaxial distraction and occipitocervical fusion. The syringomyelia width and tonsillar herniation decreased significantly, and functional scores indicated symptom relief and good outcomes. CONCLUSIONS: CVJ instability, especially the ultramovement of atlanto-condyle facets, commonly exists in II-BI as evaluated using kinematic CT. The surgical strategy of atlantoaxial distraction and occipitocervical fusion should be considered to treat such patients.

Basilar Invagination: A Tilt of the Foramen Magnum.

OBJECTIVE: Congenital basilar invagination (BI) is a craniocervical deformity marked by odontoid prolapse into the skull base. The foramen magnum angle (FMA), which is formed by the Chamberlain's line and McRae's line, has not been fully studied. The study aimed to investigate the FMA and its relationship with other craniocervical parameters. METHODS: Participants were divided into control, type A BI, and type B BI groups. Parameters included Chamberlain line violation, atlantodental interval, clivus height, clivus anteroposterior dimension, FMA, basal angle, clivo-axial angle, head and neck flexion angle, Boogard's angle, and subaxial cervical spine lordosis angle. A comparison of these parameters among the 3 groups and correlation analysis between FMA and other parameters were performed. The significance level was set at P < 0.05. RESULTS: A total of 111 controls, 111 type A BI patients, and 62 type B BI patients were enrolled. The FMAs in the control, type A BI, and type B BI groups were 6.21° (3.67°, 8.71°), 22.16° ± 6.61°, and 22.39° (17.27°, 31.08°), respectively. Correlation analysis revealed correlations between the FMA and other variables. In the 2 BI subgroups, FMA was significantly correlated with Chamberlain line violation, clivus height, clivus anteroposterior dimension, basal angle, clivo-axial angle, and Boogard's angle. CONCLUSIONS: The FMA in patients with BI was approximately 22° and approximately 6° in controls, indicating that the foramen magnum in BI had a greater tilt. As a pathological condition, FMA can reflect the degree of BI. Clivus hypogenesis is a reason for the excessive tilt of the FM.

Severe Basilar impression in osteogenesis imperfecta treated with halo gravity traction, occipitocervicothoracic fusion, foramen magnum and upper cervical decompression and expansive duroplasty: a technical note.

Osteogenesis imperfecta (OI) is a rare bone disease due to an abnormal synthesis of 1-type collagen. OI is frequently associated with basilar impression (BI), defined by the elevation of the clivus and floor of the posterior fossa with subsequent migration of the upper cervical spine and the odontoid peg into the base of the skull. Bone intrinsic fragility leading to fractures and deformity, brainstem compression and impaired CSF circulation at cranio-vertebral junction (CVJ) makes the management of these conditions particularly challenging. Different surgical strategies, including posterior fossa decompression with or without instrumentation, transoral or endonasal decompression with posterior occipito-cervical fusion, or halo gravity traction with posterior instrumentation have been reported, but evidence about best modalities treatment is still debated. In this technical note, we present a case of a 16-years-old patient, diagnosed with OI and BI, treated with halo traction, occipito-cervico-thoracic fixation, foramen magnum and upper cervical decompression, and expansive duroplasty. We focus on technical aspects, preoperative work up and postoperative follow up. We also discuss advantages and limitations of this strategy compared to other surgical techniques.

Indirect Ventral Brainstem Decompression by Posterior C1-C2 Distraction and Fixation for Basilar Invagination.

OBJECTIVE: Basilar invagination usually shows a decrease of clivus axis angle (CAA), which could give rise to progressive neural compression. Exploring a safe and effective fixation technique to achieve atlantoaxial stability and neural decompression remains necessary. In this study, we introduce a modified posterior C1-C2 distraction and fixation technique by which we obtained indirect ventral neural decompression and atlantoaxial stability in a series of patients with decreased CAA. METHODS: Thirty patients of basilar invagination were enrolled in our series. All patients underwent thin-slice computed tomography (CT) scan, magnetic resonance imaging, and dynamic plain radiography examinations before surgery, at discharge and during the follow-ups. Posterior C1-C2 facet joint release and intraoperative reduction by fastening rods were performed in all patients. The CAA was measured on midsagittal CT scans. Patients' neurologic status was evaluated by the Japanese Orthopaedic Association score. RESULTS: No neurovascular injury and serious postoperative complication occurred in all patients. Complete ventral brainstem decompression was achieved in 20 patients and partial in 10 patients. The mean postoperative CAA significantly improved to 132.6 degrees compared with the preoperative 123.6 degrees (P < 0.01). The bone fusion was confirmed in all patients on the basis of the last follow-up spine CT scans. CONCLUSIONS: Indirect ventral brainstem decompression by posterior C1-C2 distraction and fixation is a safe and effective technique for treatment of basilar invagination.

Radiological approach to basilar invagination type B: Reliability and accuracy.

OBJECTIVES: This study aims to determine the reliability of the radiological tests used in the diagnosis of basilar invagination (BI). METHODS: Patients diagnosed with type B basilar invagination, who had both magnetic resonance (MR) and computed tomography (CT) imaging between January 2014 and November 2019 were included in this retrospective reliability study. In this study, distance from odontoid apex to Chamberlain's line (OA-CL) was accepted as a reference method for the diagnosis. Forty-two BI cases and 79 controls were included. Three radiologists with different levels of experience individually evaluated OA-CL, Boogard's angle (BoA), clivo-axial angle (CXA), clivo-dens angle (CDA), and clivo-palate angle (CPA) on midsagittal CT and MR images. Statistical analysis was made with the intraclass correlation coefficient (ICC), t-test, and receiver operating characteristic (ROC) curve. RESULTS: The ICC for CT and MR were; 0.977-0.973 (OA-CL), 0.912-0.882 (BoA), 0.845-0.846 (CXA), 0.862-0.864 (CDA), and 0.762-0.747 (CPA) respectively (P < 0.001). The areas under the ROC curve were 0.977 (BoA), 0.832 (CXA), 0.852 (CDA), and 0.719 (CPA) (P < 0.001). The cut-off measures were ≥137.84° (BoA), ≤149.25° (CXA), ≤129.58° (CDA), and ≤61.83° (CPA). The diagnostic accuracies were 0.954 (BoA), 0.664 (CXA), 0.704 (CDA), 0.438 (CPA) (P < 0.001). CONCLUSIONS: OA-CL and BoA express excellent inter-rater agreement than CXA, CDA, and CPA, which are limited due to morphological variations and head spatial position. BoA is the second most reliable diagnostic test. CXA, CDA, should only be used for complementary information. CPA was found inadequate for the diagnosis of BI..

Foramen Magnum Angle: A New Parameter for Basilar Invagination of Type B.

OBJECTIVE: To describe the foramen magnum angle (FMA) as a new parameter for basilar invagination (BI) type B. METHODS: The FMA was performed on sagittal slice of magnetic resonance imaging (MRI) as a line from the hard palate to the opisthion (angle vertex), and another line from the opisthion to the basion. The MRIs from 31 participants with BI type B and 96 controls were used. Intraclass correlation coefficient, descriptive data, and receiver operating characteristic (ROC) curve were used for statistical analysis at the 95% confidence interval. RESULTS: The interobserver agreement of the FMA was 0.952. Patients with BI type B had a FMA significantly greater (25.9° ± 9.3°) than control participants (11.6° ± 4.9°) (P < 0.001). The area under the ROC curve showed a diagnostic value of 0.947. The FMA showed sensitivity 0.900 and specificity 0.854 for the cutoff criterion 17° (P < 0.001). CONCLUSIONS: The FMA had an optimal diagnostic value that provided complementary evidence to investigate BI type B.

Anterior Distraction and Reduction with Posterior Stabilization for Basilar Invagination: A Novel Technique.

BACKGROUND: Introduction of a posterior spacer for atlantoaxial joint distraction followed by posterior stabilization is a commonly performed procedure for irreducible atlantoaxial dislocation. We present a unique case in which posterior distraction was associated with increased risk of injury to the vertebral artery (VA) owing to its anomalous course, and hence a novel anterior distraction technique was used. CASE DESCRIPTION: A 45-year-old woman presented with severe neck pain for 1 month with gait imbalance and history of occipital headache for 1 year. Clinical examination revealed upper motor neuron-type findings. Hoffmann sign was positive bilaterally. Clinically, the patient had Nurick grade 4 cervical myelopathy. Magnetic resonance imaging showed basilar invagination along with Arnold-Chiari malformation and syrinx formation at C3-C4 vertebral levels. CT angiography revealed anomalous VAs directly overlying the atlanto-occipital joint. Owing to the anomalous route of the VA and unfavorable slope of facet joints, a 2-step anterior reduction followed by posterior stabilization surgery was planned. We achieved complete reduction using a 10-mm titanium cage inserted via a retropharyngeal approach. Following anterior reduction, instrumented in situ occipitocervical fusion was performed using a plate and screw construct. At 2-year follow-up, the patient is ambulating independently without gait imbalance and with successful radiologic fusion. CONCLUSIONS: The craniovertebral junction has a unique pathoanatomy, and the course of the vertebral artery is variable. Appropriate investigations, including computed tomography angiography, with adequate surgical planning will provide a desirable long-term outcome. Our novel technique has the potential to add a new dimension to the management of irreducible atlantoaxial dislocation.

A Safe and Effective Posterior Intra-Articular Distraction Technique to Treat Congenital Atlantoaxial Dislocation Associated With Basilar Invagination: Case Series and Technical Nuances.

BACKGROUND: The management of atlantoaxial dislocation (AAD) associated with basilar invagination (BI) is challenging, and traditional posterior-only approaches lack the ability to release the anterior soft tissue resulting in unsatisfactory reduction. Furthermore, vertebral artery anomalies and deformed anatomy increase surgical risks. OBJECTIVE: To introduce a safe and efficient technique to reduce congenital AAD and BI through a single-stage posterior-only approach. METHODS: A total of 65 patients with AAD and concomitant BI who had congenital osseous abnormalities were retrospectively analyzed. All patients had anterior soft tissue released through a posterior-only approach, followed by intra-facet cages implantation, cantilever correction, and instrumentation. Clinical results were measured using the Japanese Orthopedic Association (JOA) scale, and radiographic measurements included the atlanto-dental interval, the distance of odontoid tip above Chamberlain's line, clivus-canal angle (CCA), and syrinx length. Paired t-tests were used to compare preoperative and postoperative measurements. RESULTS: The mean JOA score increased from 10.98 to 14.40 at 1-yr follow-up. Complete reduction of AAD and BI was achieved in 48 patients (73.8%). The mean CCA improved from 115° preoperatively to 129° postoperatively. Reduction of syrinx size was observed in 14 patients at 1 wk and in 35 patients 1 yr after surgery. All patients achieved bony fusion. CONCLUSION: Posterior intra-articular distraction followed by cage implantation and cantilever correction can achieve complete reduction in most cases of congenitally anomalous AAD associated with BI.

Outcome of Surgery for Congenital Craniovertebral Junction Anomalies with Atlantoaxial Dislocation/Basilar Invagination: A Retrospective Study of 94 Patients.

OBJECTIVE: To evaluate the results of surgery for congenital craniovertebral junction (CVJ) anomalies with atlantoaxial dislocation (AAD)/basilar invagination (BI) and compare the results of transoral odontoidectomy and posterior fusion (TOO+PF) with only posterior fusion (PF) in patients with irreducible AAD/BI. METHODS AND RESULTS: All 94 patients with congenital CVJ anomalies with AAD/BI operated on during the 3-year study period (June 2013-May 2016) were included. Of these patients, 55 had irreducible AAD/BI and the remaining 39 had reducible AAD/BI. TOO+PF was restricted to patients (34/94; 36.2%) with irreducible AAD/BI when reduction and realignment by intraoperative C1-C2 facet joint manipulation were considered technically difficult and risky. The remaining patients with irreducible AAD/BI and all the patients with reducible AAD/BI (60/94; 63.8%) were managed with only posterior fusion. Poor preoperative Nurick grade, preoperative dyspnea/lower cranial nerve deficits, and syringomyelia were associated with significantly higher incidence of postoperative pulmonary complications. Follow-up ≥3 months (final follow-up) was available for 87 patients. Good outcome (Nurick grade 0-3) at final follow-up was noted in 90% (45/50) of the patients with irreducible AAD/BI and 91.9% (34/37) of the patients with reducible AAD/BI. Preoperative poor Nurick grade (4-5) was the only factor associated with poor outcome. No significant difference in perioperative complications, outcome, and fusion was noted between patients who underwent TOO+PF or only PF for irreducible AAD/BI. CONCLUSIONS: Many of the patients with congenital AAD/BI showed remarkable recovery after surgery. Preoperative poor Nurick grade (4-5) is associated with poor outcome. TOO+PF is a safe alternative treatment option for irreducible AAD/BI when only PF techniques are technically difficult/risky.

The Filum disease and the Neuro-Cranio-vertebral syndrome: definition, clinical picture and imaging features.

BACKGROUND: We propose two new concepts, the Filum Disease (FD) and the Neuro-cranio-vertebral syndrome (NCVS), that group together conditions thus far considered idiopathic, such as Arnold-Chiari Syndrome Type I (ACSI), Idiopathic Syringomyelia (ISM), Idiopathic Scoliosis (IS), Basilar Impression (BI), Platybasia (PTB) Retroflexed Odontoid (RO) and Brainstem Kinking (BSK). METHOD: We describe the symptomatology, the clinical course and the neurological signs of the new nosological entities as well as the changes visible on imaging studies in a series of 373 patients. RESULTS: Our series included 72% women with a mean age of 33.66 years; 48% of the patients had an interval from onset to diagnosis longer than 10 years and 64% had a progressive clinical course. The commonest symptoms were: headache 84%, lumbosacral pain 72%, cervical pain 72%, balance alteration 72% and paresthesias 70%. The commonest neurological signs were: altered deep tendon reflexes in upper extremities 86%, altered deep tendon reflexes in lower extremities 82%, altered plantar reflexes 73%, decreased grip strength 70%, altered sensibility to temperature 69%, altered abdominal reflexes 68%, positive Mingazzini's test 66%, altered sensibility to touch 65% and deviation of the uvula and/or tongue 64%. The imaging features most often seen were: altered position of cerebellar tonsils 93%, low-lying Conus medullaris below the T12L1 disc 88%, idiopathic scoliosis 76%, multiple disc disease 72% and syringomyelic cavities 52%. CONCLUSIONS: This is a paradigm shift that opens up new paths for research and broadens the range of therapeutics available to these patients.

Posterior Fossa Dimensions of Chiari Malformation Patients Compared with Normal Subjects: Systematic Review and Meta-Analysis.

BACKGROUND: Chiari malformation (CM) is defined as a herniation of cerebellar tonsils below the foramen magnum and is considered to be due to underdevelopment of posterior fossa (PF) bone components. There is overcrowding of neural structures, and normal cerebrospinal fluid circulation is frequently blocked. Although several publications exist of measurements of dimensions and volumes from bone components of PF in CM, there is no systematic review evaluating quantitatively these dimensions. The aim of this study was to evaluate PF dimensions and volumes in a meta-analysis. METHODS: An electronic search using PubMed and Google Scholar was done. Study eligibility criteria were defined by the PICO strategy, which selected patients and interventions. Studies comparing PF dimensions between patients with CM and normal subjects were selected. A meta-analysis of pooled data was done using statistical software. RESULTS: Data for pooled analysis were obtained from 12 studies. Data synthesis was based on small published sample sizes in the majority of studies. Key findings included smaller measurements of clivus, supraoccipital bone, and PF area dimensions in patients with CM compared with normal subjects. Brainstem and cerebellar length was not different between groups. CONCLUSIONS: This review provides evidence of smaller measurements of clivus, supraoccipital bone, and PF area dimensions in patients with CM compared with normal subjects. An implication of the key findings is that surgical treatment of CMs should consider the smaller dimensions of PF in planning.

Medullary Infarction Leading to Locked-In Syndrome Following Lumbar Puncture in a Patient with Basilar Invagination.

BACKGROUND: Lumbar puncture is a common procedure that can be safely performed in most patients. Certain populations may have increased risk for complications following lumbar puncture, but the significance of basilar invagination is often underappreciated. CASE DESCRIPTION: A 45-year-old woman with basilar invagination received multiple lumbar punctures in the workup of acute meningitis. Preprocedural computed tomography was obtained. Following lumbar puncture, the patient developed locked-in syndrome. Magnetic resonance imaging obtained several days later demonstrated severe compression and infarction of the medulla and inferior cerebellum by the odontoid process and ectopic cerebellar tonsils. The patient was transferred but at this point, surgical decompression was not possible. She did not regain significant neurologic function. CONCLUSIONS: Basilar invagination is a risk factor for devastating neurologic complications following lumbar puncture. Awareness of this complication and prompt recognition of its occurrence may prevent future morbidity of lumbar puncture in patients with basilar invagination.