C2 pedicle screw insertion assisted by mobilization of the vertebral artery in cases with high-riding vertebral artery.

Objective: 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).. Methods: 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. Results: 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. Conclusions: 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.

The role of clivus and atlanto-occipital lateral mass height in basilar invagination with or without atlas occipitalization.

Basilar invagination (BI) is a common deformity. This study aimed to quantitatively evaluate the height of clivus and atlanto-occipital lateral mass (LM) in patients with BI with or without atlas occipitalization (AOZ). We evaluated 166 images of patients with BI and of controls. Seventy-one participants were control subjects (group A), 68 had BI with AOZ (group B), and 27 had BI without AOZ (group C). Parameters were defined and measured for comparisons across the groups. Multiple linear regression analysis was used to test the relationship between Chamberlain's line violation (CLV) and the clivus height ratio or atlanto-occipital LM height. Based on the degree of AOZ, the lateral masses in group B were classified as follows: segmentation, incomplete AOZ, complete AOZ. From groups A to C, there was a decreasing trend in the clivus height and clivus height ratio. There was a linear negative correlation between the clivus height ratio and CLV in the three groups. Generally, the atlanto-occipital LM height followed the order of group B < group C < group A. The atlanto-occipital LM height was included only in the equations of groups B. There were no cases of atlantoaxial dislocation (AAD) in group C. There was a decreasing trend in LM height from the segmentation type to the complete AOZ type in group B. BI can be divided into three categories: AOZ causes LM height loss; Clivus height loss; Both clivus and LM height loss. The clivus height ratio was found to play a decisive role in both controls and BI group, while the atlanto-occipital LM height loss caused by AOZ could be a secondary factor in patients with BI and AOZ. AOZ may be a necessary factor for AAD in patients with congenital BI. The degree of AOZ is associated with LM height in group B.

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.

Basilar Impression: A Systematic Review and Meta-Analysis of Clinical Features, Operative Strategies, and Outcomes.

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.

Posterior Occipitocervical Fixation and Intrathecal Baclofen Therapy for the Treatment of Basilar Invagination with Klippel-Feil Syndrome: A Case Report.

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.

Revealing an Uncommon Presentation of Chiari I Malformation With Diverse Craniovertebral Anomalies in the Absence of Syringomyelia and Atlanto-Occipital Subluxation: A Case Report.

A Chiari I malformation is a frequently encountered anomaly of the posterior fossa, occurring in a notable percentage of the population. It often coexists with various other craniovertebral junction abnormalities, albeit less frequently with Klippel-Feil syndrome. Interestingly, the majority of individuals with Chiari I malformation do not exhibit any symptoms. We present a rare case of a 25-year-old male with chronic neck and occipital pain, along with progressive weakness and sensory disturbances in all four limbs, urinary urgency, and elevated left shoulder. Examination unveiled spasticity, weakness, and brisk reflexes. On extensive radiological evaluation (X-ray, CT, and MRI), findings revealed various anomalies in the craniovertebral junction, including complete atlanto-occipital assimilation, basilar invagination, and platybasia. Furthermore, cervical segmentation abnormalities indicative of Klippel-Feil syndrome were observed, along with Sprengel's deformity. MRI confirmed Chiari I malformation with tonsillar herniation and myelomalacia, as well as compression at the cervico-medullary junction. This patient underwent a surgical procedure that included transoral odontoidectomy combined with occipito-cervical fixation, after which a good clinical response was observed. It emphasizes the necessity of radiological imaging for the diagnosis of Chiari and other associated abnormalities in the craniovertebral junction.

Atlanto-occipital Assimilation with Basilar Invagination and Atlanto-occipital Dislocation Treated by Occipitocervical Fixation: A Rare Case Report.

Introduction: 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. Case Report: 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. Conclusion: 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.

A Novel Technique for Basilar Invagination Treatment in a Patient with Klippel-Feil Syndrome: A Clinical Example and Brief Literature Review.

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.

Evaluation of Craniometric Parameters and Efficacy of Posterior Surgical Intervention Types in Basilar Invagination Patients.

OBJECTIVE: Basilar invagination is one of the most frequently observed abnormalities at the craniovertebral junction, in which the odontoid process of C2 prolapses into the foramen magnum. METHODS: The current study included 27 patients who underwent surgery for basilar invagination between October 2013 and January 2023. The study group was divided into 2 groups according to basilar invagination types; type I (the presence of type A atlantoaxial instability and instability is the main pathology) and type II (the presence of type B and C atlantoaxial instability and skull base dysgenesis is the main pathology). Craniometric parameters included in the study were atlantodental interval, posterior atlantodental interval, Chamberlain's line violation, clivus-canal angle, Welcher's basal angle, and Boogaard angle. RESULTS: The mean age of the patients was 24.30 ± 14.36 years (5-57 years). Fourteen patients (51.9%) were female, and 13 patients (48.1%) were male. Ten patients (37%) had type I basilar invagination, and 17 patients (63%) had type II basilar invagination. Preoperative and postoperative atlantodental interval and Boogaard angle were significantly higher in type I basilar invagination, as preoperative and postoperative posterior atlantodental interval and clivus-canal angle were significantly higher in type II basilar invagination. There was a positive strong correlation between Chamberlain's line violation and Boogaard angle. Postoperative Chamberlain's line violation was significantly higher in occipitocervical fixation (P = 0.035). C1 lateral mass screw fixation was found more successful in Chamberlain's line violation correction than occipital plates. Occipitocervical fixation was found to be associated with higher postoperative Nurick scores (P = 0.015) and complication rates (P = 0.020). Cages applied to the C1-C2 joint space were found to be associated with higher fusion rates (P = 0.023) and lower complication rates (P = 0.024). CONCLUSIONS: In the present study, it was found that C1-C2 fixation was more successful in correcting craniometric parameters and had lower complication rates than occipitocervical fixation. In appropriate patients, it was determined that cage application increased the success rates of the operations.

A Global Bibliometric and Visualization Analysis of Craniovertebral Junction Bony Abnormalities Based on VOSviewer and Citespace.

OBJECTIVE: Recent years have witnessed a rapidly growing interest in CVJ bony abnormalities, and a qualitative and quantitative analysis of relevant literatures is necessary. This study aims to identify and summarize the published articles related to craniovertebral junction bony abnormalities, to analyze and visualize the current research trends and major contributors. METHODS: We collected data from Web of Science, excluding certain article types. Two researchers screened articles for relevance. Data were organized with EndnoteX9, and analyzed using VOSviewer and CiteSpace for co-authorship, co-occurrence, keyword burst, and co-citation analyses to identify research trends and collaborations. RESULTS: A total of 2,776 articles were included, revealing an increasing trend in annual publications of CVJ bony abnormalities. The USA was the leading country. King Edward Memorial Hospital was the most prolific institution, and Seth GS Medical College had the most citations. The Spine is the most popular journal with the highest number of publications and citations. Professor Goel Atul from India emerged as the most influential pioneer in this field. Keyword analysis highlighted surgical techniques, diagnosis, and anatomy as the primary research hotspots and Fixation, Placement, and Basilar invagination gradually become the new research trend. However, there is a relative weakness in basic research and epidemiology. CONCLUSIONS: This study provides valuable insights into the current research trends and critical contributors in CVJ bony abnormalities, guiding evidence-based decisions and fostering international collaborations to advance knowledge in this field.

Neurological outcome following delayed traction and fixation in severe tetraparesis consecutive to posterior decompression for Chiari malformation: a case report.

BACKGROUND: Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered. CASE DESCRIPTION: We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement. DISCUSSION AND CONCLUSION: This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.

Transcervical, retropharyngeal odontoidectomy - Anatomical considerations.

Context: Anterior craniocervical junction lesions have always been a challenge for neurosurgeons. Presenting with lower cranial nerve dysfunction and symptoms of brainstem compression, decompression is often required. While posterior approaches offer indirect ventral brainstem decompression, direct decompression via odontoidectomy is necessary when they fail. The transoral and endoscopic endonasal approaches have been explored but come with their own limitations and risks. A novel retropharyngeal approach to the cervical spine has shown promising results with reduced complications. Aims: This study aims to explore the feasibility and potential advantages of the anterior retropharyngeal approach for accessing the odontoid process. Methods and Surgical Technique: To investigate the anatomical aspects of the anterior retropharyngeal approach, a paramedian skin incision was performed below the submandibular gland on two cadaveric specimens. The subcutaneous tissue followed by the platysma is dissected, and the superficial fascial layer is opened. The plane between the vascular sheath laterally and the pharyngeal structures medially is entered below the branching point of the facial vein and internal jugular vein. After reaching the prevertebral plane, further dissection cranially is done in a blunt fashion below the superior pharyngeal nerve and artery. Various anatomical aspects were highlighted during this approach. Results: The anterior, submandibular retropharyngeal approach to the cervical spine was performed successfully on two cadavers highlighting relevant anatomical structures, including the carotid artery and the glossopharyngeal, hypoglossal, and vagus nerves. This approach offered wide exposure, avoidance of oropharyngeal contamination, and potential benefit in repairing cerebrospinal fluid fistulas. Conclusions: For accessing the craniocervical junction, the anterior retropharyngeal approach is a viable technique that offers many advantages. However, when employing this approach, surgeons must have adequate anatomical knowledge and technical proficiency to ensure better outcomes. Further studies are needed to enhance our anatomical variations understanding and reduce intraoperative risks.

Craniometric parameters for the evaluation of platybasia and basilar invagination on magnetic resonance imaging: a reproducibility study / Parâmetros craniométricos para platibasia e invaginação basilar na ressonância magnética: estudo de reprodutibilidade

Abstract Objective: The present study aims to perform a reproducibility study of the clivus-canal angle (CCA), Welcker's basal angle (WBA), and the distance from the odontoid process to Chamberlain's line (DOCL) on magnetic resonance imaging (MRI). Materials and Methods: Two medical students and two radiologists respectively evaluated 100 and 50 consecutive MRI scans of adult skulls, selected randomly. Each examiner, working independently and blinded to the previous results, performed readings for each patient on two different occasions. Measurements were performed in T1-weighted sequences acquired in the midsagittal plane. The levels of intraobserver reproducibility and interobserver agreement were evaluated by calculating the intraclass correlation coefficients (ICCs) and the corresponding 95% confidence intervals. Results: The mean values obtained by the examiners were 150° for the CCA, 130° for the WBA, and 2.5 mm for the DOCL. The ICC for interobserver agreement was 0.980, 0.935, and 0.967, for the CCA, WBA, and DOCL, respectively, for the students, compared with 0.977, 0.941, and 0.982, respectively, for the radiologists, and 0.980, 0.992, and 0.990, respectively, for all of the examiners together. In the analysis of intraobserver agreement, the ICC ranged from 0.929 to 0.959 for the CCA, from 0.918 to 0.964 for the WBA, and from 0.918 to 0.981 for the DOCL. Conclusion: The measurement of the CCA, WBA, and DOCL appears to show excellent intraobserver reproducibility and interobserver agreement on MRI.

Resumo Objetivo: Realizar um estudo de reprodutibilidade do ângulo clivocanal (ACC), ângulo basal de Welcker (ABW) e distância do odontoide à linha de Chamberlain (DOLC) em ressonância magnética (RM). Materiais e Métodos: Quatro examinadores, dois graduandos de medicina e dois radiologistas, avaliaram, respectivamente, 100 e 50 indivíduos adultos submetidos a RM de crânio, consecutiva e aleatoriamente. Cada um realizou duas leituras para cada paciente em diferentes ocasiões, de forma cega e independente. As análises de concordância intraobservador e interobservador foram realizadas pelo coeficiente de correlação intraclasse (CCI), com intervalo de confiança de 95%. Resultados: As medidas médias, considerando todos os examinadores, foram: ACC = 150°, ABW = 130°, DOLC = 2,5 mm. A análise interobservador entre os estudantes revelou CCI de 0,980, 0,935 e 0,967 para ACC, ABW e DOLC, respectivamente, e para os radiologistas, CCI de 0,977, 0,941 e 0,982, respectivamente. A análise interobservador entre estudantes e radiologistas revelou CCI de 0,980, 0,992 e 0,990, respectivamente. Em relação à análise intraobservador, as medidas do ACC tiveram CCI variando entre 0,929 e 0,959, ABW entre 0,918 e 0,964 e DOLC entre 0,918 e 0,981. Conclusão: ACC, ABW e DOLC obtiveram excelentes reprodutibilidades intraobservador e interobservador na RM.

Basilar invagination associated with chiari malformation type I: A literature review

Basilar invagination (BI) and Chiari malformation type I (CM-I) are very important anomalies that introduce instability and compression in the occipitocervical transition region and have complex clinical characteristics. These anomalies vary according to the affected structures. The present study revises current knowledge regarding the anatomy, anatomo-physiology, clinical manifestations, and radiological findings of these entities and the associated surgical treatment approaches. A bibliographic survey was performed through a search in the Medline, PubMed, SciELO, Science and LILACS databases. When associated, these craniovertebral malformations result in neurological deficits due to neural parenchyma compression; however, the presence of microtraumas due to repetitive lesions caused by the bulb and cervical marrow instability has been highlighted as a determinant dysfunction. Surgical treatment is controversial and has many technical variations. Surgery is also challenging due to the complex anatomical characteristics and biomechanics of this region. Nevertheless, advances have been achieved in our understanding of related mechanisms, and compression and atlantoaxial instability are considered key elements when selecting the surgical approach.

Direct Intraoperative Reduction of Basilar Invagination Associated with Atlantoaxial Subluxation ­ Technical Note

In the present article, we describe a technique of direct intraoperative reduction of congenital atlantoaxial subluxation associated with severe basilar invagination and atlas assimilation. It consists of a wide dissection of the craniovertebral junction, exposing the region between the superior facet of C2 and the occiput (described as a "pseudoarticulation" by some authors). After exposure, a self-rotating small dissector or Kobe is used bilaterally, pushing C2 anteriorly and inferiorly, with reduction of the dislocation, followed by craniovertebral fixation. The technique is safe and eliminates the need for an anterior approach to the odontoid reduction, as well as the need to dissect the C1­2 joint.

Neste artigo, descrevemos a técnica de redução intraoperatória direta de subluxação atlantoaxial congênita associada a invaginação basilar grave e assimilação do atlas. Ela consiste em ampla dissecção da junção craniovertebral, expondo a região entre a face superior da C2 e o osso occipital (descrita como uma "pseudoarticulação" por alguns autores). Após exposição, um descolador pequeno ou Kobe é usado bilateralmente com rotação própria, deslocando C2 anterior e inferiormente, com redução da luxação, seguido de fixação craniovertebral. A técnica é segura e elimina a necessidade de uma abordagem anterior para a ressecção do odontoide, além de evitar a dissecação da junta C1­C2.

Management of a Complex Basilar Invagination Case with Multiple Revision Surgeries ­ Case Report

We describe a Basilar Invagination (BI) case with craniocervical instability and many previous failure surgeries and poor wound coverage. The patient had been submitted to a large posterior fossa craniectomy (which greatly limited the availability of an adequate area for bone fixation) and showed a poor quality of the surgical wound in the posterior craniocervical region. We performed an occipito-cervical fixation, using the bone overlying the torculla as a point of cranial fixation. Craniocervical realignment was achieved by the use of distractive maneuvers with occipital rods, followed by coverage of the hardware via a pedicled longitudinal trapeze myocutaneous flap. We used local ribs removed from the region where the myocutaneous flap was harvested as autologous bone grafts for craniocervical fusion. Post-operatively, the patient was placed in a halo-vest for three months. The patient improved substantially after the procedure, recovered some muscular strength and experienced total relief of her pain. We hereby discuss the surgical strategy used for treating this complex case in details, with illustrative pictures.

Descrevemos caso de paciente com diagnóstico de invaginação basilar e instabilidade crânio cervical com múltiplas cirurgias prévias e deiscência de ferida operatória. Devido a falha de osso na escama occipital, assim como da cobertura cutânea adequada, realizamos realinhamento craniocervical, com descompressão indireta anterior, fixação occipitocervical na região da tórcula e cobertura da pele com flap miocutâneo longitudinal pediculado de trapézio. As costelas removidas da região do retalho miocutâneo foram transferidas para serem usadas como enxerto autólogo de osso para fusão craniocervical. No pós-operatório, a paciente utilizou um halo-vest por 3 meses. No presente artigo, apresentamos nuances ilustrados de manobras para realinhamento craniocervical por via posterior na invaginação basilar, bem como estratégias para otimizar a artrodese e o fechamento cutâneo.

Incidence of basilar invagination in patients with tonsillar herniation ? a case control craniometrical study / Incidência da invaginação basilar com heriação tonsilar ? estudo da craniometria do tipo caso-controle

A retrospective case-control study based on craniometrical evaluation was performed to evaluate the incidence of basilar invagination (BI). Patients with symptomatic tonsillar herniation treated surgically had craniometrical parameters evaluated based on CT scan reconstructions before surgery. BI was diagnosed when the tip of the odontoid trespassed the Chamberlain’s line in three different thresholds found in the literature: 2, 5 or 6.6 mm. In the surgical group (SU), the mean distance of the tip of the odontoid process above the Chamberlain’s line was 12 mm versus 1.2 mm in the control (CO) group (p<0.0001). The number of patients with BI according to the threshold used (2, 5 or 6.6 mm) in the SU group was respectively 19 (95%), 16 (80%) and 15 (75%) and in the CO group it was 15 (37%), 4 (10%) and 2 (5%).

Realizamos estudo retrospectivo tipo caso-controle baseado na avaliação craniométrica para avaliar a incidência da Invaginação Basilar (IB). Pacientes com herniação tonsilar sintomática tratada cirurgicamente foram avaliados quanto a parâmetros craniométricos obtidos em reconstrução de TC antes da cirurgia. IB foi diagnosticada quando a ponta do odontóide passava acima da linha de Chamberlain em 2, 5 ou 6,6 mm. No grupo cirúrgico (CI), a distância média da ponta do odontóide acima da linha de Chamberlain foi de 12 mm versus 1,2 mm no grupo controle (CO) (p<0.0001). O número de pacientes com IB conforme o critério diagnóstico usado (2, 5 ou 6,6 mm) foi de 19 (95%), 16 (80%) e 15 (75%) no grupo CI, respectivamente, contra 15 (37%), 4 (10%) e 2 (5%) no grupo CO. Pacientes com herniação tonsilar tinham maior incidência de IB comparados ao grupo controle.

Alterações radiográficas de coluna cervical em artrite reumatoide / Radiographic changes of cervical spine in rheumatoid arthritis

INTRODUÇÃO: O envolvimento da coluna cervical é comum na artrite reumatoide (AR). OBJETIVO: Estudar a prevalência das alterações radiológicas de coluna cervical em pacientes com AR e sua associação com perfil clinico e sorológico da doença. MÉTODOS: Analisaram-se as radiografias de coluna cervical em perfil neutro hiperextensão, hiperflexão e transoral de 80 pacientes com AR para presença de subluxação atlanto-axial (LAA), invaginação basilar (IB) e instabilidade subaxial (ISA). Dados de perfil demográfico, clínico (nódulos, pneumonite intersticial, síndrome Sjögren secundária, uso de medicamentos etc.) e sorológico (FR, anti-CCP e FAN) foram obtidos por revisão de prontuários. RESULTADOS: Havia alguma alteração de eixo de coluna cervical em 26/80 (32,5%); em 12/80 (15%) havia LAA; em 6/80(7,5%) existia IB; em 13/80 (16,2%) existia ISA. Erosões em odontoide foram vistas 16/80 (20,0%). As alterações do eixo cervical estavam associadas com idade de início da doença e duração da mesma (P = 0,03 e 0,02, respectivamente). Não se encontrou associação das alterações em coluna cervical com raça, gênero, nódulos, pneumonite intersticial, Sjögren secundário, FR, FAN ou anti-CCP. Pacientes com alterações do eixo cervical apresentavam mais erosões de odontoide (P = 0,03). CONCLUSÕES: Alterações radiológicas em eixo de coluna cervical são comuns e aparecem mais frequentemente em indivíduos com diagnóstico mais precoce de AR e maior tempo de doença.

INTRODUCTION: The involvement of the cervical spine is a common feature of rheumatoid arthritis (RA). OBJECTIVE: To study the prevalence of radiographic changes of the cervical spine in patients with RA and their association with clinical and serological profiles of the disease. METHODS: We analysed lateral (neutral position, hyperextension, hyperflexion) and transoral views of cervical spine radiographs from 80 individuals with RA to investigate the presence of atlanto-axial subluxation (AAS), basilar invagination (BI), and subaxial instability (SAI). Demographic, clinical (nodules, interstitial pneumonitis, secondary Sjögren's syndrome, medications etc.), and serologic (rheumatoid factor - RF, cyclic citrullinated peptide antibody - anti-CCP, and antinuclear factor - ANF) data were obtained from the clinical records. RESULTS: Cervical spine misalignments were identified in 26/80 (32.5%) participants; AAS occurred in 12/80 (15%) participants, BI in 6/80 (7.5%), and SAI in 13/80 (32.5%). Odontoid erosions were identified in 16/80 (20.0%) participants. Cervical spine misalignment exhibited associations with age at onset and disease duration (P = 0.03 and 0.02, respectively). No associations were identified between the cervical spine changes and the participants' ethnicity or gender, presence of nodules, interstitial pneumonitis, secondary Sjögren's syndrome, RF, ANF, or anti-CCP. The participants with cervical spine misalignment exhibited higher frequencies of odontoid erosion (P = 0.03). CONCLUSIONS: Cervical spine misalignment was a common radiographic finding and occurred more frequently in participants with earlier onset and longer length of RA.

Transoral approach to the craniovertebral junction

The transoral approach provides a safe exposure to lesions in the midline and the ventral side of the craniovertebral junction. The advantages of the transoral approach are 1) the impinging bony pathology and granulation tissue are accessible only via the ventral route; 2) the head is placed in the extended position, thus decreasing the angulation of the brainstem during the surgery; and 3) surgery is done through the avascular median pharyngeal raphe and clivus. We analyzed the clinical effects of odontoidectomy after treating 38 patients with basilar invagination. The anterior transoral operation to treat irreducible ventral compression in patients with basilar invagination was performed in 38 patients. The patientsÆ ages ranged from 34 to 67 years. Fourteen patients had associated Chiari malformation and eight had previously undergone posterior decompressive surgery. The main indication for surgery was significant neurological deterioration. Symptoms and signs included neck pain, myelopathy, lower cranial nerve dysfunction, nystagmus and gait disturbance. Extended exposure was performed in 24 patients. The surgery was beneficial to the majority of patients. There was one death within 10 days of surgery, due to pulmonary embolism. Postoperative complications included two cases of pneumonia, three cases of oronasal fistula with regurgitation and one cerebrospinal fluid leak. In patients with marked ventral compression, the transoral approach provides direct access to the anterior face of the craniovertebral junction and effective means for odontoidectomy.

O acesso transoral é uma via direta e segura às lesões situadas na linha média e na face anterior da junção craniocervical. As vantagens do acesso transoral são as seguintes:1) a compressão óssea e o tecido de granulação localizam-se anteriormente e são accessíveis pela via anterior; 2) a cabeça do paciente é colocada em extensão, diminuindo a angulação do tronco cerebral durante a cirurgia; e 3) a cirurgia é feita através de um plano avascular na linha média faríngea e clivo. Analisamos os resultados obtidos após odontoidectomia por via transoral em 38 pacientes portadores de invaginação basilar. Trinta e oito pacientes com compressão ventral da junção craniocervical foram submetidos a odontoidectomia por via transoral. A idade dos pacientes variou de 34 a 67 anos. Quatorze pacientes apresentavam associação com malformação de Chiari tipo I e 8 já haviam sido submetidos à cirurgia descompressiva por via posterior. A maioria dos pacientes apresentou nucalgia, mielopatia, déficits dos nervos cranianos baixos, nistagmo, e distúrbio da marcha. Em 24 pacientes foi necessário ampliar o acesso transoral através de miotomia do palato mole, ou osteotomia do palato duro ou maxilotomia. A cirurgia proporcionou melhora dos sintomas na maioria dos pacientes. Um paciente faleceu no pós-operatório imediato por causa de embolia pulmonar. Dois pacientes tiveram pneumonia, três apresentaram fístula oronasal com regurgitação, e um teve fístula liquórica. Em pacientes com compressão ventral irredutível da junção craniocervical, a via transoral proporcionou uma abordagem direta e ampla ao processo odontoide.