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Osteogenesis Imperfecta is a rare, hereditary bone condition with an incidence of 1/15,000–20,000. Symptoms include bone fragility, long bone deformity, scoliosis, hypermobility, alongside secondary features such as short stature, basilar invagination, pulmonary and cardiac complications, hearing loss, dentinogenesis imperfecta and malocclusion. Osteogenesis Imperfecta can have a large impact on the child and their family; this impact starts immediately after diagnosis. Fractures, pain, immobility, hospital admissions and the need for equipment and adaptations all influence the health-related quality of life of the individual and their family. This narrative review article aims to examine the impact the diagnosis and management of osteogenesis imperfecta has on the health-related quality of life of a child. It will touch on the effect this may have on the quality of life of their wider family and friends and identify strategies to optimise health-related quality of life in this population. Optimising health-related quality of life in children with Osteogenesis Imperfecta is often a complicated, multifaceted journey that involves the child, their extended family, school, extracurricular staff and numerous health professionals. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to analyze failed posterior fossa decompression (PFD) in patients with basilar invagination and atlantoaxial dislocation (BI‐AAD). Revision surgery in these patients is challenging and has been rarely reported. In addition, the anatomical variations of the vertebral artery increase the risk of revision surgery. Here, we introduce the implementation of a new type of one‐stage posterior revision surgery, whose difficulties and effects are summarized. Methods: A total of 21 patients with BI‐AAD who underwent PFD were retrospectively analyzed in our center from November 2017 to April 2021. The revision surgery in all patients was performed through the posterior approach. The Japanese Orthopaedic Association (JOA) score and the Short Term 12 (SF‐12) score were employed to evaluate the clinical symptoms and health status. The distance from the tip of the odontoid to Chamberlain's line (DCL), the atlantodental interval (ADI), the clivus‐canal angle (CCA), the diameter of the subarachnoid space (DSS), and the craniovertebral junction triangular area (CTA) were assessed radiographically. The pre‐ and postoperative results were compared by paired t test. Results: The data of 21 consecutive patients were reviewed, with an average follow‐up period of 28 ± 14 months. Postoperative imaging showed effectively reduced compression of BI‐AAD. No implant failure or neurovascular injury occurred. Eleven patients had vertebral artery abnormalities, but none had vertebral artery injury. All patients had evidence of bone fusion on the CT scan images within a 12‐month follow‐up period. The JOA and SF‐12 scores were significantly improved 1 year postoperatively (p < 0.001). Conclusion: Posterior surgery using the technique of interarticular distraction, fusion with cage grafting, and fixation is a safe and effective revision surgery to treat patients with basilar invagination and atlantoaxial dislocation who failed PFD, which will result in good outcome. [ABSTRACT FROM AUTHOR]

Basilar invagination has been classified into two types by Goel: Type A is defined mechanical instability of the atlantoaxial joint with upward displacement of the odontoid process while Type B is characterized by stable atlantoaxial joints. This study reviews the association between radiological features and symptomatology and prognosis of two types of basilar invagination for better clinical management. A retrospective analysis was conducted including 141 patients diagnosed with basilar invagination who underwent surgical treatment from January 2016 to December 2020. The neurological function was assessed by the JOA scores, and Short-Form 12 scores. Logistic univariate and multivariate analyses were performed to predict prognostic risk factors. Type A patients (21/101, 20.8%) with more cases of dizziness, lower preoperative JOA scores and SF-12 PCS scores (JOA, 13.0 vs. 13.9, P = 0.042; SF-12 PCS, 37.48 vs. 38.42, P = 0.034) compared to type B (2/40, 5%) (P = 0.022). Type B (22/40, 55%) with more cases of ataxia than type A (35/101, 34.7%) (P = 0.026). Patients with type B demonstrated a significantly higher improvement rate in SF-12 PCS than type A (P = 0.018). Further logistic regression revealed that onset age ≥ 45 years (OR 4.654, 95% CI 1.645–13.165; p = 0.004) and basal angle ≥ 125° (OR 28.139, 95% CI 1.090-726.239; p = 0.044) were independent risk factors for type A and type B, respectively. Type A patients with more cases of dizziness, and type B patients with more cases of ataxia, can achieve better long-term prognosis following clinical intervention. [ABSTRACT FROM AUTHOR]

To evaluate the effect of the basilar invagination (BI) type B on cervical spine. 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% CI. Participants with BI type B showed shorter clivus length (CLI: 25.7 ± 7.3 mm); 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). The deformation of skull base in the BI of type B caused, on average, a hyperlordosis of almost 30° in the C2-C6 segment. This change was approximately 17° in the C2-C4, with the clivus hypoplasia being a risk factor for cervical hyperlordosis. [ABSTRACT FROM AUTHOR]

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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Abstract Background Congenital craniovertebral deformity, including basilar invagination (BI) and atlantoaxial instability (AAI), are often associated with three-dimensional (3D) deformity, such as C1-2 rotational deformity, craniocervical kyphosis, C1 lateral inclination, among other abnormalities. Effective management of these conditions requires the restoration of the 3D alignment to achieve optimal reduction. Recently, 3D printing technology has emerged as a valuable tool in spine surgery, offering the significant advantage of allowing surgeons to customize the prosthesis design. This innovation provides an ideal solution for precise 3D reduction in the treatment of craniovertebral deformities. Objective This study aims to describe our approach to individualized computer-simulated reduction and the design of C1-2 intra-articular 3D printed porous titanium alloy cages for the quantitative correction of craniovertebral junction deformities. Methods A retrospective analysis was conducted on patients with craniovertebral deformities treated at our institution using individualized 3D-printed porous titanium alloy cages. Preoperative CT data were used to construct models for 3D realignment simulations. Cage designs were tailored to the simulated joint morphology following computer-assisted realignment. Preoperative and postoperative parameters were statistically analyzed. Results Fourteen patients were included in the study, with a total of 28 3D-printed porous titanium alloy cages implanted. There were no cases of C2 nerve root resection or vertebral artery injury. All patients experienced symptom relief and stable implant fixation achieved in all cases. No implant-related complications were reported. Conclusion The use of individualized computer-simulated reduction and the design of C1-2 intra-articular 3D printed porous titanium alloy cage facilitates precise 3D realignment in patients with craniovertebral deformities, demonstrating effectiveness in symptom relief and stability.

Objective Recent studies indicate that 3 morphological types of atlanto-occipital joint (AOJ) exist in the craniovertebral junction and are associated with type II basilar invagination (BI) and atlanto-occipital instability. However, the actual biomechanical effects remain unclear. This study aims to investigate biomechanical differences among AOJ types I, II, and III, and provide further evidence of atlanto-occipital instability in type II BI. Methods Models of bilateral AOJ containing various AOJ types were created, including I-I, I-II, II-II, II-III, and III-III models, with increasing AOJ dysplasia across models. Then, 1.5 Nm torque simulated cervical motions. The range of motion (ROM), ligament and joint stress, and basion-dental interval (BDI) were analyzed. Results The C0–1 ROM and accompanying rotational ROM increased progressively from model I-I to model III-III, with the ROM of model III-III showing increases between 27.3% and 123.8% indicating ultra-mobility and instability. In contrast, the C1–2 ROM changes were minimal. Meanwhile, the stress distribution pattern was disrupted; in particular, the C1 superior facet stress was concentrated centrally and decreased substantially across the models. The stress on the C0–1 capsule ligament decreased during cervical flexion and increased during bending and rotating loading. In addition, BDI gradually decreased across the models. Further analysis revealed that the dens showed an increase of 110.1% superiorly and 11.4% posteriorly, indicating an increased risk of spinal cord impingement. Conclusion Progressive AOJ incongruity critically disrupts supportive tissue loading, enabling incremental atlanto-occipital instability. AOJ dysplasia plays a key biomechanical role in the pathogenesis of type II BI.

Background: Osteoradionecrosis (ORN) of the upper cervical spine is a rare but severe complication of head and neck cancer radiotherapy. To raise awareness of this condition, we describe a patient with a history of nasopharyngeal carcinoma who developed ORN of the upper cervical spine and review the published literature reporting surgical management. Case presentation: A 59-year-old female patient with persistent neck pain for one month and limited range of neck motion who had undergone radiotherapy for nasopharyngeal carcinoma with a total dose of 69.96 Gy 15 years ago presented to our hospital. The patient underwent endoscopic transnasal and transoral resection of the odontoid process and C1 anterior arch, combined with occipitocervical fusion. To better understand surgical management of ORN of the upper cervical spine, the literature published in the PubMed, Ovid MEDLINE, and Embase databases was reviewed. Our patient experienced alleviation of cervical pain and did not exhibit any postoperative complications. Since 2005, 11 cases of surgical management of ORN of the upper cervical spine (including the present case) have been published. Basilar invagination and/or atlantoaxial subluxation were observed in 4 /11 cases. Endoscopic procedures were performed in 4/11 cases, and occipitocervical fusion was performed in 8 /11 cases. Conclusion: Endoscopic transnasal and transoral resection of the odontoid process and C1 anterior arch is a safe and effective treatment option for ORN of the upper cervical spine. Occipitocervical fusion is useful in patients with basilar invagination and atlantoaxial subluxation. [ABSTRACT FROM AUTHOR]

Aim and Background: This study aimed to investigate the relationship between cervical spinal alignment and the center of gravity (COG) of the head in patients with Chiari malformation (CM) compared to healthy individuals. CM is characterized by the herniation of posterior fossa structures through the foramen magnum, potentially affecting head positioning and craniovertebral junction biomechanics. Understanding these biomechanical changes is crucial for improving diagnostic and treatment strategies. Materials and Methods: This retrospective study included 102 CM patients and 71 healthy controls. Radiological measurements were obtained from cervical X-rays, with seven reference points used to calculate angles related to head positioning and cervical curvature. Angular parameters, including cranial incidence (CI), cranial slope (CS), cranial tilt (CT), C7 slope (C7S), and spinocranial angle (SCA), were analyzed to determine correlations with the COG of the head. Statistical analyses were performed using t -tests, ROC analysis, and Pearson/Spearman correlation tests. Results: CM patients had significantly higher CI, CT, STT, and SCA angles compared to controls (P < 0.05), indicating an anterior displacement of the head's COG. The CS angle was lower in CM patients (P < 0.05), reflecting a more flexed head position. No significant differences were found in C7S and C2T angles between groups, suggesting similar cervical curvature. ROC analysis demonstrated high sensitivity and specificity of the angular measurements for diagnosing CM. Conclusion: CM patients exhibit distinct biomechanical alterations, including an anterior shift of the COG and a more flexed head position. These findings highlight the potential of angular measurements as noninvasive diagnostic tools for CM. Future studies should explore the implications of these biomechanical changes on CM progression and treatment outcomes. [ABSTRACT FROM AUTHOR]

Case: A 47-year-old man presented with neck pain, restricted neck movements, along with involvement of facial and hypoglossal nerve. On the basis of clinico-radiological correlation, the patient was diagnosed with craniovertebral junction tuberculosis and was started on antitubercular therapy (ATT). Failing the conservative trial, the patient was operated and occipitocervical fusion was done with bone grafting. After 12 months of ATT intake, he showed complete recovery of the facial and hypoglossal nerve, with complete resolution of instability pain. Conclusion: Craniovertebral junction tuberculosis is uncommon, and its manifestation with cranial nerves involvement is even more rare. Meticulous clinico-radiological assessment should be done to diagnose and plan the treatment. Early recognition and prompt initiation of ATT, along with timely surgical intervention, when necessary, are crucial for optimal management and outcomes. [ABSTRACT FROM AUTHOR]

Background: Chiari malformation type 1 (CM1) remains a complex neurosurgical condition with ongoing debate regarding its optimal management. Methods: This narrative review examines key controversies surrounding the pathophysiology, surgical indications, and treatment strategies for CM1. Results: We highlight the challenges posed by the wide spectrum of CM variants and the evolving understanding of its association with syringomyelia, basilar invagination, and craniovertebral instability. Emerging surgical techniques, including minimally invasive approaches and the use of new technologies such as endoscopes and exoscopes, are evaluated for their potential to improve outcomes. Recent consensus guidelines are also discussed. Conclusions: The need for individualized treatment plans for CM1 is emphasized, with special focus put on the connection between novel pathophysiological insights, technological advancements and opportunities for a more nuanced surgical management. Further research is necessary to establish solid foundations for more individualized treatments. [ABSTRACT FROM AUTHOR]

Background: Congenital craniovertebral deformity, including basilar invagination (BI) and atlantoaxial instability (AAI), are often associated with three-dimensional (3D) deformity, such as C1-2 rotational deformity, craniocervical kyphosis, C1 lateral inclination, among other abnormalities. Effective management of these conditions requires the restoration of the 3D alignment to achieve optimal reduction. Recently, 3D printing technology has emerged as a valuable tool in spine surgery, offering the significant advantage of allowing surgeons to customize the prosthesis design. This innovation provides an ideal solution for precise 3D reduction in the treatment of craniovertebral deformities. Objective: This study aims to describe our approach to individualized computer-simulated reduction and the design of C1-2 intra-articular 3D printed porous titanium alloy cages for the quantitative correction of craniovertebral junction deformities. Methods: A retrospective analysis was conducted on patients with craniovertebral deformities treated at our institution using individualized 3D-printed porous titanium alloy cages. Preoperative CT data were used to construct models for 3D realignment simulations. Cage designs were tailored to the simulated joint morphology following computer-assisted realignment. Preoperative and postoperative parameters were statistically analyzed. Results: Fourteen patients were included in the study, with a total of 28 3D-printed porous titanium alloy cages implanted. There were no cases of C2 nerve root resection or vertebral artery injury. All patients experienced symptom relief and stable implant fixation achieved in all cases. No implant-related complications were reported. Conclusion: The use of individualized computer-simulated reduction and the design of C1-2 intra-articular 3D printed porous titanium alloy cage facilitates precise 3D realignment in patients with craniovertebral deformities, demonstrating effectiveness in symptom relief and stability. [ABSTRACT FROM AUTHOR]

Objective: Recent studies indicate that 3 morphological types of atlanto-occipital joint (AOJ) exist in the craniovertebral junction and are associated with type II basilar invagination (BI) and atlanto-occipital instability. However, the actual biomechanical effects remain unclear. This study aims to investigate biomechanical differences among AOJ types I, II, and III, and provide further evidence of atlanto-occipital instability in type II BI. Methods: Models of bilateral AOJ containing various AOJ types were created, including I-I, I-II, II-II, II-III, and III-III models, with increasing AOJ dysplasia across models. Then, 1.5 Nm torque simulated cervical motions. The range of motion (ROM), ligament and joint stress, and basion-dental interval (BDI) were analyzed. Results: The C0–1 ROM and accompanying rotational ROM increased progressively from model I-I to model III-III, with the ROM of model III-III showing increases between 27.3% and 123.8% indicating ultra-mobility and instability. In contrast, the C1–2 ROM changes were minimal. Meanwhile, the stress distribution pattern was disrupted; in particular, the C1 superior facet stress was concentrated centrally and decreased substantially across the models. The stress on the C0–1 capsule ligament decreased during cervical flexion and increased during bending and rotating loading. In addition, BDI gradually decreased across the models. Further analysis revealed that the dens showed an increase of 110.1% superiorly and 11.4% posteriorly, indicating an increased risk of spinal cord impingement. Conclusion: Progressive AOJ incongruity critically disrupts supportive tissue loading, enabling incremental atlanto-occipital instability. AOJ dysplasia plays a key biomechanical role in the pathogenesis of type II BI. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the efficacy of calvarial graft (CG) in craniovertebral fusion procedures in children at a single single center. Methods: Paediatric patients in whom CG had been used as the sole construct, or to augment a semi-rigid construct were identified from a prospective operative database. Age, underlying diagnosis and clinical presentation were obtained from review of the electronic patient record. The primary outcome was bony fusion confirmed on CT. Additional outcome measures were donor site morbidity and need for further surgery. Results: From 82 paediatric CVJ procedures, CG was used in 15 patients with a mean age of 4.1 (± 3.52) years. Aetiology comprised skeletal dysplasia (n = 12), congenital anomaly of segmentation (n = 1) and cervical trauma (n = 2). Myelopathy was the most common clinical finding (9/15), followed by cervical pain (3/15). The indications for surgery comprised atlanto-axial subluxation (8/15), basilar invagination with compression (2/15), and cervicomedullary compression without instability but deemed at risk of instability following decompression (4/15). CG was used in three scenarios: (i) CG + wire only (n = 10); (ii) CG + semirigid instrumentation (n = 3); (iii) CG to augment rigid instrumented fixation (n = 2). In 13 patients a Halo-body Jacket was used peri-operatively. At a mean time of 4.4 months following surgery, 80% of cases had radiological evidence of fusion. Conclusion: Full thickness calvarial bone graft is readily available, has good structural integrity and is associated with minimal donor site morbidity. CG should be considered for use as a sole construct, or to augment semi-rigid constructs when instrumented fixation is precluded. [ABSTRACT FROM AUTHOR]

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

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). 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. 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. 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. [ABSTRACT FROM AUTHOR]

In 1994, the use of interfacet spacer placement was for joint distraction, reduction, and fusion to supplement atlantoaxial or occipitocervical fixation. Here, we present a unique case of bilateral atlantoaxial interfacet fixation using cervical facet cages (CFC) in a pediatric patient with basilar invagination. In addition, we review the literature on atlantoaxial facet fixation. We present a 12-year-old boy with Wiedemann-Steiner syndrome who presented with multiple episodes of sudden neck jerking, described as in response to a sensation of being shocked, and guarding against neck motion, found to have basilar invagination with cervicomedullary compression. He underwent an occiput to C3 fusion with C1-C2 CFC fixation. We also conducted a literature review identifying all publications using the following keywords: "C1" AND "C2" OR "atlantoaxial" AND "facet spacer" OR "DTRAX." The patient demonstrated postoperative radiographic reduction of his basilar invagination from 6.4 to 4.1 mm of superior displacement above the McRae line. There was a 4.5 mm decrease in the atlantodental interval secondary to decreased dens retroflexion. His postoperative course was complicated by worsening of his existing dysphagia but was otherwise unremarkable. His neck symptoms completely resolved. We illustrate the safe use of CFC for atlantoaxial facet distraction, reduction, and instrumented fixation in a pediatric patient with basilar invagination. Review of the literature demonstrates that numerous materials can be safely placed as a C1-C2 interfacet spacer including bone grafts, titanium spacers, and anterior cervical discectomy and fusion cages. We argue that CFC may be included in this arsenal even in pediatric patients. [ABSTRACT FROM AUTHOR]

Atlanto-occipital assimilation is a congenital craniovertebral junction malformation where the partial or complete fusion of the atlas and occiput occurs. Atlanto-occipital assimilation can be associated with numerous complications, including basilar invagination, atlantoaxial subluxation, Chiari malformation, congenital vertebral bodies fusion, and cord compression. As a result, vigilant identification and accurate reporting of atlanto-occipital assimilation are essential. The purpose of this review article is to discuss the numerous complications and associations of atlanto-occipital assimilation, such that an accurate diagnosis could be made for this commonly missed pathology. [ABSTRACT FROM AUTHOR]

Background: The Craniovertebral Junction (CVJ) is prone to various pathologies, including instability and congenital anomalies. Understanding these conditions and their management strategies is critical for effective treatment. Materials and Methods: A systematic search was conducted in Science Direct and PubMed databases following PRISMA guidelines. Inclusion criteria encompassed studies addressing craniovertebral instability and associated pathologies. Six systematic investigations were assessed for methodological quality. Data extraction involved 702 patients with CVJ issues, among which 129 had related conditions, while 279 displayed normal CVJ. Surgical interventions encompassed various techniques such as C1-C2 fixation, posterior decompression, and screw placements. Results: Among 702 patients studied, atlantoaxial subluxation, basilar invagination, and odontoid fractures were observed in 129 cases. Surgical treatments showed favorable outcomes, with fusion achieved within a year post-surgery for both C1-C2 fixation techniques and posterior decompression strategies. Studies highlighted successful outcomes in cases of cervical myelopathy, especially with early occipitocervical fusion. Conclusion: Managing atlantoaxial instability remains a debated topic, with varying success rates observed in different surgical interventions. Recommendations emphasize the importance of stabilization techniques and imaging modalities for effective preoperative planning and postoperative care. However, limitations in available data underscore the need for further research to refine treatment strategies for better patient outcomes in this complex area of spinal pathology. [ABSTRACT FROM AUTHOR]

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Study Design. Retrospective study. Objective. To evaluate the feasibility of C2 pedicle screw fixation with the "in-out-in" technique in patients with basilar invagination (BI). Summary of Background Data. The "in-out-in" technique is a fixation technique in which the screw enters the vertebrae through the parapedicle. The technique has been used in upper cervical spine fixation. However, anatomic parameters associated with the application of this technique in patients with BI are unclear. Materials and Methods. We measured the C2 pedicle width (PW), the distance between the vertebral artery (VA) and the transverse foramen (VATF), the safe zone, and the limit zone. The lateral safe zone is the distance from the medial/lateral cortex of the C2 pedicle to the VA (LPVA/MPVA), and the medial safe zone is the distance from the medial/lateral cortex of the C2 pedicle to the dura (MPD/LPD). The lateral limit zone is the sum of LPVA/MPVA and VATF (LPTF/MPTF), and the medial limit zone is the distance from the medial/lateral cortex of the C2 pedicle to the spinal cord (MPSC/LPSC). PW, LPVA, MPVA, and VATF were measured on the reconstructed CT angiography. PW, MPD, LPD, MPSC, and LPSC weremeasured onMRI.We define a width greater than 4mmas safe for screw. The t-test was used to compare the parameters between male and female, left and right sides in all patients, and PW in CTA and MRI data in the same patient. For intrarater reliabilities, interclass correlation coefficients were calculated. Results. A total of 154 patients (49 CTA, 143 MRI) were included. The average PW, LPVA, MPVA, LPTF, MPTF, MPD, LPD, MPSC, and LPSC were 5.30 mm, 1.28 mm, 6.60 mm, 2.45 mm, 8.94 mm, 2.09 mm, 7.07 mm, 5.51 mm, and 10.48 mm, respectively. Furthermore, in patients with PW < 4 mm, 53.6% of MPVA, 86.2% of LPTF, and all limit zones were larger than 4 mm. Conclusions. In patients with BI, there is sufficient space medially and laterally to the C2 pedicle for partial screw encroachment to achieve "in-out-in" fixation, even if the pedicle is small. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

To evaluate the difficulty of reduction of congenital atlantoaxial dislocation with or without os odontoideum or basilar invagination based on computed tomography (CT) quantitative analysis. From March 2018 to December 2022, the CT features of 108 patients with atlantoaxial dislocation with or without os odontoideum or basilar invagination were analyzed. Quantitative scores were defined according to imaging features, including sloping of the lateral mass; osteophyte between the lateral mass joint; ball-and-socket deformity of the lateral mass joint; vertical interlocking of the lateral mass joint; callus between the lateral mass joint; and atlanto-odontoid joint hyperplasia, blocking, or fusion. Grades were calculated according to the sum of points of the atlanto-odontoid joint and lateral mass joints, as follows: I, 0–1 points; Ⅱ, 2–3 points; Ⅲ, 4–6 points; IV, 7–10 points. After 1 week of bidirectional cervical traction, CT scans were performed, and atlantodens interval and vertical distance from dens to Chamberlain line were measured. The vertical reduction rate, horizontal reduction rate, and overall reduction rate of atlantoaxial dislocation were calculated. The vertical distance from dens to Chamberlain line values after traction were significantly reduced compared with before traction, including grades I, II, III, and IV. The overall reduction rates were 85.1% ± 11.8%, 65.8% ± 8.3%, 45.0% ± 8.5%, and 38.4% ± 13.0% respectively, after 1 week of bidirectional cervical traction. The CT quantitative score system is an effective noninvasive evaluation to judge the reduction difficulty of atlantoaxial dislocation with or without os odontoideum or basilar invagination. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Trigeminal neuralgia (TN) is a headache characterized by paroxysmal episodes of intense pain in the facial region. TN can occur secondary to structural mechanisms, such as vascular compression of the trigeminal nerve root. Basilar invagination (BI) is a malformation of the craniovertebral junction characterized by invagination of the odontoid process of the axis through the foramen magnum into the posterior fossa, and 1% of cases may present associated TN. This article presents a clinical case of TN secondary to BI and vascular compression of the trigeminal nerve root, which responded only to treatment with botulinum toxin type A. CASE REPORT: A 34-year-old patient with a clinical presentation consistent with TN for approximately 12 years. The symptoms were debilitating and impacted the quality of life, culminating in constant insomnia, severe depression, and suicidal thoughts. Treatment with botulinum toxin type A was fundamental in managing this patient’s pain. CONCLUSION: The case reported here demonstrated the therapeutic success of treatment with botulinum toxin type A in a complex and refractory case of pain syndrome.

Abstract Background The posterior cranial fossa (PCF) and the foramen magnum (FM) are the critical anatomical components of the craniovertebral junction region, which comprise and transmit numerous vital neurovascular structures. So, a fundamental knowledge of the basic radiological anatomy of PCF and FM is of paramount importance in the evaluation of associated pathologies and approaching these areas surgically. The aim of this study is to describe different linear and angular craniometric parameters of PCF, FM and surrounding territory based on reconstructed computed tomography (CT) images. Material and methods This study was conducted in our tertiary care hospital in northern India from the period of January 2023 to June 2023 on 120 patients, and CT screening was done for the head and spine region following a history of head injury. Results In this study, 120 patients were included, of whom 50.83% (n = 61) were females and 49.17% (n = 59) were males. Age ranged from 18 to 70 years with mean age of 43.5 ± 14.08 years. The mean values for linear craniometric parameters of PCF were statistically nonsignificant for different age groups. Statistically significant differences were found for twinning line (TL) (p

We evaluate the efficacy, safety and indications of single stage posterior release, reduction, and fixation of basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD). Seventeen patients with BI and IAAD consecutively underwent one-stage release, reduction, and fixation by a posterior approach from July 2000 to June 2015 were followed up for at least 12 months. There were 8 males. Mean age was 56 35.2 ± 13.8 years (range 12–56). The clinical symptoms and signs of the patients were recorded. Pre- and postoperative imaging examinations were performed. Neurological function was assessed using the Japanese Orthopedic Association (JOA) and Ranawat scores. Average follow-up time was 47.4 months (12–97 months). The JOA score increased from preoperative 4–10 (8.06 ± 2.52) to postoperative 13–16 (15.20 ± 0.62). The preoperative Chamberlain line, McRae line, Wackenheim line, atlantodens interval, and cervico medullary angle were 12.52 ± 5.17 mm, 6.59 ± 3.04 mm, 6.96 ± 4.32 mm, 9.88 ± 1.93 mm, and 115.35 ± 12.40°, respectively. The postoperative values were 2.0 ± 3.67 mm, −3.06 ± 1.85 mm, −1.76 ± 2.88 mm, 1.17 ± 1.18 mm, and 136.76 ± 11.44°, respectively. One-stage release, reduction, and fixation for patients with BI and IAAD through a posterior approach is safe and efficient. [ABSTRACT FROM AUTHOR]

Occipitalization of atlas is an osseous anomaly of the craniovertebral junction, which occurs at the base of the skull in the region of the foramen magnum. It is the fusion of first cervical vertebra with the occipital bone of the skull. A 16-year-old patient reported with chief complaint of irregularly placed teeth. Routine lateral cephalogram assessment revealed the presence of fusion between first cervical vertebrae (C-1) and base of skull. The radiographic investigation revealed the presence of fusion along with the clinical findings of short neck, short face, cleft palate, missing teeth, and pain during neck extension and flexion. Such cases of craniocervical anomaly can cause orthopedic and neurological problems like abnormal posture of head, restricted neck movements, cervical pain, pain in extremities, wasting of muscles, and convulsions. The comprehensive radiographic assessment of all structures visible in cephalogram of orthodontic patient can help in identification of such anomalies and sensitize the patients about symptomatic or asymptomatic condition. [ABSTRACT FROM AUTHOR]

Introduction: Cranio‐cervical anomalies are significant complications of osteogenesis imperfecta (OI), a rare bone fragility disorder that is usually caused by mutations in collagen type I encoding genes. Objective: To assess cranio‐cervical anomalies and associated clinical findings in patients with moderate‐to‐severe OI using 3D cone beam computed tomography (CBCT) scans. Methods: Cross‐sectional analysis of CBCT scans in 52 individuals with OI (age 10–37 years; 32 females) and 40 healthy controls (age 10–32 years; 26 females). Individuals with a diagnosis of OI type III (severe, n = 11), type IV (moderate, n = 33) and non‐collagen OI (n = 8) were recruited through the Brittle Bone Disorders Consortium. Controls were recruited through the orthodontic clinic of the University of Missouri‐Kansas City (UMKC). Results: OI and control groups were similar in mean age (OI: 18.4 [SD: 7.2] years, controls: 18.1 [SD: 6.3] years). The cranial base angle was increased in the OI group (OI: mean 148.6° [SD: 19.3], controls: mean 130.4° [SD: 5.7], P =.001), indicating a flatter cranial base. Protrusion of the odontoid process into the foramen magnum (n = 7, 14%) and abnormally located odontoid process (n = 19, 37%) were observed in the OI group but not in controls. Low stature, expressed as height z‐score (P =.01), presence of DI (P =.04) and being male (P =.04) were strong predictors of platybasia, whereas height z‐score (P =.049) alone was found as positive predictor for basilar impression as per the Chamberlain measurement. Conclusion: The severity of the phenotype in OI, as expressed by the height z‐score, correlates with the severity of cranial base anomalies such as platybasia and basilar impression in moderate‐to‐severe OI. Screening for cranial base anomalies is advisable in individuals with moderate‐to‐severe OI, with special regards to the individuals with a shorter stature and DI. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Study Design: Retrospective cohort study. Objective: To investigate the effect of HRVA on the intrapedicular accuracy of C2PS placement through the freehand method in patients with BI and analyse the possible risk factors for C2PS malpositioning. Method: A total of 91 consecutive patients with BI who received 174 unilateral C2PS placements through the freehand method were retrospectively included. The unilateral pedicles were assigned to the HRVA and non-HRVA groups. The primary outcome was the intrapedicular accuracy of C2PS placement in accordance with the Gertzbein–Robbins scale. Moreover, the risk factors that possibly affected intrapedicular accuracy were assessed. Results: The rate of intrapedicular accuracy in C2PS placement in patients with BI was 23.6%. Results showed that the non-HRVA group had remarkably higher rates of optimal and clinically acceptable C2PS placement than the HRVA group. Nevertheless, the HRVA group exhibited similar results for grade B classification as the non-HRVA group. Moreover, in the HRVA and non-HRVA groups, the most common direction of screw deviations was the lateral direction. Furthermore, the multivariate analyses showed that the obliquity of the lateral atlantoaxial joint in the sagittal plane ≥15°, and that in the coronal plane ≥ 20°, isthmus height < 4.3 mm, and distance from the skin to the spinous process ≥ 2.8 cm independently contributed to a high rate of screw malpositioning in BI patients. Conclusion: The presence of HRVA in BI patients contributed to the high rate of malpositioning in C2PS placement via the freehand method. However, the rates of intrapedicular accuracy in patients with BI with and without HRVA were considerably low. [ABSTRACT FROM AUTHOR]

Background: The posterior cranial fossa (PCF) and the foramen magnum (FM) are the critical anatomical components of the craniovertebral junction region, which comprise and transmit numerous vital neurovascular structures. So, a fundamental knowledge of the basic radiological anatomy of PCF and FM is of paramount importance in the evaluation of associated pathologies and approaching these areas surgically. The aim of this study is to describe different linear and angular craniometric parameters of PCF, FM and surrounding territory based on reconstructed computed tomography (CT) images. Material and methods: This study was conducted in our tertiary care hospital in northern India from the period of January 2023 to June 2023 on 120 patients, and CT screening was done for the head and spine region following a history of head injury. Results: In this study, 120 patients were included, of whom 50.83% (n = 61) were females and 49.17% (n = 59) were males. Age ranged from 18 to 70 years with mean age of 43.5 ± 14.08 years. The mean values for linear craniometric parameters of PCF were statistically nonsignificant for different age groups. Statistically significant differences were found for twinning line (TL) (p < 0.0001), McRae's line (< 0.0001), clivus length (< p < 0.0001), internal occipital protuberence -opisthion line (p = 0.01), Klaus' index (p < 0.0001), height of posterior fossa (h) (p < 0.0001), h/TL (p = 0.028), when these values were compared for the genders. The measurements of FM transverse diameter, anteroposterior diameter and area were 27.12 ± 1.42 mm (range 23.6–30.1 mm), 30.99 ± 2.23 mm (range 27.6–35.8 mm) and 691.32 ± 30.35 mm2 (range 632.7–777.7 mm2). The values of clivus canal angle (p = 0.038) and clivoodontoid angle (p = 0.012) were statistically significant when compared for different age groups. The values of Boogard's angle (p = 0.021) and tentorial slope (p = 0.031) were statistically significant when these were compared for the genders. Conclusions: This study described almost all the linear and angular craniometric parameters used in the morphometric analysis of PCF and FM. The findings of this study provide valuable data regarding linear and angular craniometric parameters of PCF and FM which can redefine reference values. [ABSTRACT FROM AUTHOR]

Osteogenesis Imperfecta (OI) is a genetic disease characterized by osteopenia and bone fragility in which the craniocervical junction is also affected. This is of special relevance due to the high prevalence in anomalies described in the literature as follows: basilar invagination, basilar impression, and platybasia. Methods. We analyzed 19 lateral skull radiographs and 14 magnetic resonance images (MRIs) of 28 patients with OI in which eight linear and five angular measurements were plotted to determine the existence of craniocervical junction anomalies and compare them with 38 lateral skull radiographs and 28 MRIs performed on age-matched healthy controls. Results. From the reference values obtained from the control sample for each age group, we established the limit value at which pathology could be suspected. Some of the variables studied showed a clear trend associated with growth. More than half of the patients (60.71%) presented an anomaly in the skull base. Conclusions. According to the diagnostic criteria used and taking +2.5 SD as the limit value, 10.71% of the patients had basilar invagination, 35.71% had basilar impression, and 39.29% had platybasia, the latter being the most common finding. [ABSTRACT FROM AUTHOR]

We describe our experience with management of basilar invagination (BI) with the atlantoaxial dislocation (C1/C2) joint reduction technique, including posterior atlantoaxial internal fixation. From 2008 to 2018, eleven patients with atlantoaxial dislocation (AAD) and BI underwent surgical reduction using C1/C2 the joint reduction technique with a fibular graft/peek cage placement followed by C1 lateral mass/C2 pedicle screw fixation. In two cases that we originally planned to perform C1/C2 joint reduction, occiput-C2 pedicle screw fixation was performed instead due to intraoperative challenges. Post-operative course and surgical complications will be discussed. A total of 13 patients, with an average age of 30.46 ± 13.23 years (range 12–57), were operated. In one patient, iatrogenic vertebral artery injury occurred without any neurological complication. JOA score improved from 10.45 ± 1.128 to 15.0 ± 1.949 (p < 0.0001, paired t-test). All radiological indices were improved (p at least < 0.001). No construct failure was seen in any of the patients with C1-2 facet joint distraction technique during follow-up, and no additional anterior decompression surgery was required. C1/C2 joint reduction technique with fibular graft/cervical PEEK cage of BI patients together with AAD seems to be an effective and safe surgical method of treatment. [ABSTRACT FROM AUTHOR]

This article discusses the use of endoscopic endonasal odontoidectomy as a treatment for pediatric patients with ventral brainstem compression caused by congenital skeletal dysplasia. The study involved four pediatric patients who underwent posterior instrumented fusion followed by endoscopic endonasal odontoid resection. The patients experienced symptoms such as dysphagia, dysphonia, gait instability, extraocular movement dysfunction, and tongue numbness. The procedure was successful in achieving brainstem decompression and improving or resolving preoperative symptoms. The article highlights the challenges of performing this procedure in pediatric patients, including narrow nasopharyngeal anatomy and the need for preoperative planning and specialized instruments. [Extracted from the article]

This article, published in the Journal of Neurological Surgery, discusses the measurement of the superior and inferior limits of the endoscopic endonasal approach (EEA) to the craniovertebral junction. The EEA is a minimally invasive method for decompressing this area, with lower morbidity rates compared to traditional approaches. The study aimed to measure these limits purely through the endonasal trans-choanal approach, without the use of septostomy. The results showed that the rhinopalatine line (RPL) predicted the inferior limit more accurately than other reference lines, making it feasible to perform surgery on conditions such as basilar invagination or lower clival lesions without damaging the septum or other nasal structures. [Extracted from the article]

This article presents a retrospective review of pediatric patients who underwent endoscopic endonasal surgery (EES) for skull base pathologies at a large tertiary pediatric hospital between 1999 and 2022. The study included 243 patients with a median age of 14.2 years, with a male predominance. Various pathologies were treated, including juvenile nasopharyngeal angiofibroma, craniopharyngioma, chordoma, pituitary adenoma, encephalocele, Rathke cleft cyst, and basilar invagination or odontoid pannus. Most patients were treated with EES alone, and the overall rate of postoperative complications was 14%. CSF leak was the most common complication. The study concludes that EES is an effective approach for pediatric skull base pathologies, with lower neurovascular complication rates compared to traditional open approaches. [Extracted from the article]

This article discusses the use of extended endoscopic endonasal approaches (EEA) in the treatment of anterior cranio-vertebral junction (aCVJ) disorders. The study conducted a retrospective analysis of 38 patients who underwent EEA for various conditions, including bulbo-medullary compression, CVJ malformations, and non-healed odontoid fractures. The results showed improvement in all cases, with successful decompression and bone fusion. The EEA was found to be a promising alternative to traditional surgical approaches, offering reduced invasiveness and improved maneuverability. [Extracted from the article]

Abstract Background: The Craniovertebral Junction (CVJ) is prone to various pathologies, including instability and congenital anomalies. Understanding these conditions and their management strategies is critical for effective treatment. Materials and Methods: A systematic search was conducted in Science Direct and PubMed databases following PRISMA guidelines. Inclusion criteria encompassed studies addressing craniovertebral instability and associated pathologies. Six systematic investigations were assessed for methodological quality. Data extraction involved 702 patients with CVJ issues, among which 129 had related conditions, while 279 displayed normal CVJ. Surgical interventions encompassed various techniques such as C1-C2 fixation, posterior decompression, and screw placements. Results: Among 702 patients studied, atlantoaxial subluxation, basilar invagination, and odontoid fractures were observed in 129 cases. Surgical treatments showed favorable outcomes, with fusion achieved within a year post-surgery for both C1-C2 fixation techniques and posterior decompression strategies. Studies highlighted successful outcomes in cases of cervical myelopathy, especially with early occipitocervical fusion. Conclusion: Managing atlantoaxial instability remains a debated topic, with varying success rates observed in different surgical interventions. Recommendations emphasize the importance of stabilization techniques and imaging modalities for effective preoperative planning and postoperative care. However, limitations in the available data underscore the need for further research to refine treatment strategies for better patient outcomes in this complex area of spinal pathology. Keywords: Craniovertebral junction, atlantoaxial instability, Basilar invagination, fusion, CVJ pathologies

This case report discusses the presentation of an 8-year-old boy with atlanto-axial rotatory dislocation (AARD) and basilar invagination, a rare complication of inflammatory bowel disease unclassified (IBDU). Basilar invagination is an anomaly of the craniovertebral junction that can be associated with other abnormalities. The patient initially underwent evaluation at a local hospital, where no pathologies were found. However, further examination revealed signs of spondylarthritis and basilar invagination. The patient underwent surgical stabilization and received biological treatment, resulting in a significant reduction in symptoms. This case highlights the importance of thorough examination and imaging methods in pediatric cases of torticollis associated with IBDU to prevent life-threatening complications. [Extracted from the article]

Sleep apnoea is common in patients with Basilar Invagination with Arnorld Chiari Malformation (ACM). Various studies have shown its incidence in the range of 60-70% among such patients. Most of the studies have shown improvement in sleep disturbances after decompressive surgeries for Chiari Malformations. There is no report of postoperative deterioration due to sleep apnoea in these patients. Authors report two cases of basilar invagination associated with ACM and Platybasia, who deteriorated probably due to worsening of pre-existing sleep disorders on 3rd and 7th postoperative days after their surgeries, despite clinico-radiological improvements during their early post-operative courses. Authors discuss literature related to sleep apnoea in basilar invagination associated with Chiari Malformations and share precautions, which are relevant and should be undertaken in such patients especially during early post-operative periods to avoid alarming complication which may occur even in experienced hands. [ABSTRACT FROM AUTHOR]

Basilar invagination (BI) is characterized by rostral dislocation of the cervical spine toward the skull base. The craniometrics of the skull base have shown significant differences among craniocervical junction malformations. The sphenoid bone is the center of the skull base; however, no study has evaluated this bone in cases of BI. This was a cross-sectional study of MRI databanks from two institutions of the author's practice between 1985 and 2020. The craniometrics of the sphenoid bone were measured in BI patients and controls. Fifty-eight MRIs were selected, including 28 BI patients and 30 controls. The mean sphenoid crest-clivus length was 32.66 ± 4.7 mm in the BI group and 29.98 ± 3.0 mm in the control group (p = 0.01). The mean sphenoid planum-top of Dorsum sellae length was 28.53 ± 3.7 mm in the BI group and 26.45 ± 3.2 mm in the control group (p = 0.02). The mean tuberculum sellae–sphenoid floor height was 18.52 ± 4.4 mm in the BI group and 21.32 ± 2.9 mm in the control group (p = 0.00). The mean sella turcica–sphenoid floor height was 10.35 ± 3.8 mm in the BI group and 12.24 ± 3.5 mm in the control group (p = 0.05). The mean clivus length was 29.81 ± 6.3 mm in the BI group and 40.86 ± 4.2 mm in the control group (p = 0.00). The mean sphenoid length was 58.34 ± 7.4 mm in the BI group and 67.31 ± 6.0 mm in the control group (p = 0.00). The mean sphenoid angle was 116.33 ± 8.7° in the BI group and 112.36 ± 6.9° in the control group (p = 0.05). The BI sphenoid bone has shorter vertical dimensions and longer horizontal measures. This morphology promotes a flattening of the sphenoid angle. The sphenoid bone is significantly altered in BI, favoring the congenital hypothesis in the pathophysiology of this disease. [ABSTRACT FROM AUTHOR]