A new etiology of nontraumatic C0-C1-C2-Complex instability - abnormality of musculus rectus capitis posterior minor: a case report.

PURPOSE: This study presents an abnormality of the musculus rectus capitis posterior minor (RCPmi) as a new etiological factor for nontraumatic sagittal plane instability in the C0-C1-C2-complex, with a focus on identifying the absence or atrophy of RCPmi on both sides. METHODS: A 36-year-old male patient presented with recurring neck pain (VAS 8/10) and tingling paresthesia in the entire left hand over a six-month period, without significant neurological deficits. Radiated arm pain was not reported. Imaging examinations revealed sagittal plane instability in the C0-C1-C2-complex, spinal canal stenosis (SCS), and myelopathy at the C1 level. Subsequently, a dorsal C0-1 reposition and fusion with laminectomy were performed. RESULTS: The congenital absence or atrophy of RCPmi, leading to the lack of cephalad-rearward traction on the C1-tuberculum-posterius, induced a developmental failure of the C1 posterior arch. Consequently, the oblate-shaped C1 posterior arch lost support from the underlying C2 posterior arch and the necessary cephalad-rearward traction throughout the patient's 36-year life. This gradual loss of support and traction caused the C1 posterior arch to shift gradually to the anterior side of the C2 posterior arch, resulting in a rotational subluxation centered on the C0/1 joints in the sagittal plane. Ultimately, this led to SCS and myelopathy. Traumatic factors were ruled out from birth to the present, and typical degenerative changes were not found in the upper cervical spine, neck muscles, and ligaments. CONCLUSION: In this case, we not only report the atrophy or absence of RCPmi as a new etiological factor for nontraumatic sagittal plane instability in the C0-C1-C2-complex but also discovered a new function of RCPmi. The cephalad-rearward traction exerted by RCPmi on the C1 posterior arch is essential for the development of a normal C1 anterior-posterior diameter.

Challenges in Occipitocervical Fusion in a 9-Month-Old Infant: A Case Report and Review of Literature.

ABSTRACT: Traumatic cranio-cervical junction injuries in infants are rare and require early surgical stabilization. In view of the unique anatomy of the occipitocervical junction in infants, the creation of a fusion construct that is both safe and biomechanically sound is challenging. A 9-month-old male infant involved in an accident presented with weakness in both upper limbs. Imaging revealed a displaced odontoid synchondrosis fracture with combined occipitocervical and atlantoaxial dissociation. Due to the unstable nature of the injury, occiput to C2 arthrodesis using dual distal radius locking plates and autologous full-thickness parietal region calvarial bone graft was performed. Minerva jacket was applied. Computed tomography (CT) scan obtained at 12 weeks demonstrated evidence of bony fusion. Combined injuries to the occipitocervical and atlantoaxial joint in an infant can be fatal. A thorough assessment of multiple imaging modalities helps to make an early and accurate diagnosis.

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.

Relationship between clinical parameters and malformations in dogs diagnosed with atlanto-axial instability.

Atlanto-axial instability is a common disease that affects toy-breed dogs. Most cases of atlanto-axial instability are congenital. Furthermore, patients with atlanto-axial instability are predisposed to other concurrent diseases. Therefore, this study aimed to retrospectively determine the presence of concurrent diseases in cases with atlanto-axial instability using imaging data and analyze the relationship between clinical parameters and the incidence of complex malformations. The clinical data and imaging findings of 41 toy-breed dogs diagnosed with atlanto-axial instability were analyzed using their medical records and imaging data. Occipital dysplasia (17/27), atlanto-occipital overlapping (22/34), dens dysplasia (27/41), Chiari-like malformation (8/34), syringomyelia (5/34), lateral ventricular enlargement (20/36), and intracranial arachnoid cyst (5/35) were observed in patients with atlanto-axial instability. The body weight of the patients in the groups with atlanto-occipital overlapping and lateral ventricular enlargement was lower than that of those in the groups without these diseases (1.78 ± 0.71 vs 2.71 ± 1.15 kg, P = 0.0269, 1.60 ± 0.40 vs 2.75 ± 1.08 kg, P = 0.001, respectively). Furthermore, when the correlation between the total number of concurrent diseases and the age at onset and body weight was examined, it became clear that lower body weight was associated with the incidence of a greater number of concurrent diseases. Thus, the findings of this study suggest that toy-breed dogs are more likely to present with complex malformations and should be evaluated carefully with additional examinations and treatment methods.

"Letter to the editor: traumatic atlantoaxial rotatory fixation in adults: a systematic review of published cases".

This critique evaluates a recent study on adult traumatic atlantoaxial rotatory fixation (AARF), focusing on its strengths, weaknesses, and suggestions for future research. The study provides a comprehensive examination of the anatomical and biomechanical complexities of the C1-C2 articulation, shedding light on the rare nature of adult traumatic AARF and common injury mechanisms. It categorizes AARF based on the atlanto-dental interval (ADI) and dislocation severity, aiding clinicians in assessing injury severity and treatment planning. Furthermore, the study explores conservative and surgical management approaches, offering valuable insights into treatment decision-making and outcomes. However, limitations such as its retrospective nature, reliance on reported cases, lack of standardized protocols, and limited sample size may constrain the generalizability of findings. Future research should prioritize prospective, multicenter studies with standardized protocols, collaborative efforts among institutions, and innovative techniques to advance our understanding and management of adult traumatic AARF.

Clinical characteristics of upper cervical spine injuries in children: a retrospective case series.

OBJECTIVE: Pediatric cervical spine injuries (CSI) can be devastating, and children < 8 years are particularly at risk for upper CSI given unique anatomical differences. Diagnosis of these injuries can be delayed due to variable clinical presentations and a paucity of existing literature. The authors aimed to characterize the spectrum of pediatric upper CSI. METHODS: This was a retrospective, single-center case series of trauma patients aged < 16 years who were assessed at a level I pediatric trauma center and diagnosed with upper CSI between 2000 and 2020. Patients were included if they had evidence of bony or ligamentous injury from the occiput to C2 on imaging or autopsy. Data were obtained from manual chart review and analyzed using descriptive statistics. RESULTS: In total, 502 patients were screened and 202 met inclusion criteria. Of these, 31 (15%) had atlanto-occipital (AO) joint distractions, 10 (5%) had atlanto-axial (AA) joint distractions, 31 (15%) had fractures of C1-2, and 130 (64%) had ligamentous injury without joint distraction. Of the patients with AO injury, 15 patients had complete dislocation. They presented as hemodynamically unstable with signs of herniation and 14 died (93%). In contrast, 16 had incomplete dislocation (subluxation). They usually had stable presentations and survived with good outcomes. Of the patients with AA injury, 2 had complete dislocation, presented with arrest and signs of herniation, and died. In contrast, 8 patients with subluxation mostly presented as clinically stable and all survived with little residual disability. The most common fractures of C1 were linear fractures of the lateral masses and of the anterior and posterior arches. The most common fractures of C2 were synchondrosis, hangman, and odontoid fractures. Overall, these patients had excellent outcomes. Ligamentous injuries frequently accompanied other brain or spine injuries. When these injuries were isolated, patients recovered well. CONCLUSIONS: Among upper CSI, AO and AA joint injuries emerged as particularly severe with high mortality rates. Both could be divided into complete dislocations or incomplete subluxations, with clear clinical differences and the former presenting with much more severe injuries. Lateral cervical spine radiography should be considered during resuscitation of unstable trauma patients to assess for these CSI subtypes. Fractures and ligamentous injuries were clinically heterogeneous, with presentations and outcomes depending on severity and associated injuries.

Atlantoaxial rotatory subluxation following surgical treatment of a cervical teratoma in a child: a case report.

Atlantoaxial rotatory subluxation (AARS), which is characterised by an abnormal alignment of the atlantoaxial joint, is rarely reported after oral and maxillofacial surgery. A four-year-old girl developed AARS after neck surgery. The child initially had treatment for one month in a timely manner. Follow ups revealed reduced symptoms of neck pain and the previous tilt disappeared after serial treatment. This case aimed to increase awareness of AARS and provide a reference for oral and maxillofacialsurgeons.

Dens Growth After Surgical Fixation in Children with Morquio-Brailsford Disease.

Mucopolysaccharidosis type IVA is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase. Mucopolysaccharidosis type IVA is multisystemic disease with significant spinal involvement and atlantoaxial instability leading to neural compression and significant morbidity. Dens hypoplasia is a common feature of this condition. In this study we demonstrate that after spinal fixation, there is new growth of dens in significant proportion of patients, suggesting atlantoaxial instability as one of the major driving forces of lack of development of dens in this condition.

Design of a novel lateral mass screw-plate system for the treatment of unstable atlas fractures: a finite element analysis.

BACKGROUND: Osteosynthesis of unstable atlas fractures preserves joint motion and therefore has a distinct advantage over a range of treatment procedures. To prevent the potential disadvantages associated with osteosynthesis, a new atlas lateral mass screw-plate (LMSP) system has been designed. However, the biomechanical role of using the LMSP system in atlas internal fixation is not known. The aim of this study was to compare the biomechanical stability of a new LMSP with traditional posterior screw and rod (PSR) fixation techniques on the occipitocervical junction (C0-C2) through finite element analysis. METHODS: A nonlinear C0-C2 finite element model of the intact upper cervical spine was developed and validated. The unstable model using the PSR system was then compared with the model using the LMSP system for fixation. A vertical load of 40 N was applied to the C0 to simulate head weight, while a torque of 1.5 Nm was applied to the C0 to simulate flexion, extension, lateral bending, and axial rotation. RESULTS: The range of motion of both systems was close to the intact model. Compared with the LMSP system model, the PSR system model increased flexion, extension, lateral bending, and axial rotation by 4.9%, 3.0%, 5.0%, and 29.5% in the C0-C1 segments, and 4.9%, 2.7%, 2.4%, and 22.6% in the C1-C2, respectively. In flexion, extension, and lateral bending motion, the LMSP system model exhibited similar stress to the PSR system model, while in axial rotation, the PSR system model exhibited higher stress. CONCLUSIONS: The findings of our study indicate that the two tested system models provide comparable stability. However, better stability was achieved during axial rotation with the LMSP system, and in this system, the maximum von Mises stress was less than that of the PSR one. As the atlantoaxial joint functions primarily as a rotational joint, the use of the LMSP system may provide a more stable environment for the joint that has become unstable due to fracture.

[Advances in the technology of surgical treatment for atlantoaxial dislocation].

The etiology and pathological morphology of atlantoaxial dislocation (AAD) are complex. Based on the pathological anatomical characteristics of AAD, combined with clinical techniques, it is essential to formulate reasonable classification criteria and develop corresponding treatment strategies for different types of AAD. The satisfactory outcome of surgical treatment for AAD can only be achieved through the comprehensive application of various atlantoaxial reduction techniques, tension band releasing techniques, internal fixation and fusion techniques. This article discusses the latest advancements in surgical treatment techniques for AAD, thoroughly explores treatment strategies based on different types of AAD, and analyzes the practicality and effectiveness of clinical classification and treatment strategies. The posterior atlantoaxial facet releasing and distraction compression reduction technique may pose challenges to traditional treatment strategies in the future. In the development of surgical treatment techniques for AAD, microspinal surgical techniques may play a significant role in improving surgical methods and enhancing treatment outcomes.

Early Outcomes of Bone Autografting in the Bilateral Atlantoaxial Joints Applied in Posterior Fusion Surgery.

OBJECTIVE: Cable-dragged reduction and cantilever beam internal fixation can provide promising results in the treatment of atlantoaxial dislocation or instability. However, bilateral atlantoaxial joints bone autografting has not been conducted in this technique. We aim to evaluate the safety and effectiveness of bilateral atlantoaxial joints bone autografting in posterior cable-dragged reduction and cantilever-beam internal fixation. METHODS: In this retrospective study, we included 14 patients with a minimum 24-month follow-up from December 2019 to September 2020. The granular bone harvested from the iliac crest was packed into the bilateral atlantoaxial joints of 14 patients in posterior cable-dragged reduction and cantilever-beam internal fixation. X-ray imaging and cervical computed tomography (CT) were performed during follow-up. The time required for bone fusion was recorded. The clinical outcomes were evaluated using the JOA scores, NDI, and VAS scores. Mann-Whitney U test, the chi-squared test, or the Fisher exact test were used to compare the two groups regarding patient characteristics, clinical outcomes, bone fusion rates, and cervical sagittal alignment. RESULTS: The operations were successfully performed in all patients without any intraoperative complications. The mean operation time was (169.64 ± 20.91) minutes, and the intraoperative blood loss was (130.71 ± 33.62) mL. All patients received satisfactory reductions and firm bony fusion at the final follow-up. The fusion rates were 64.29% in the atlantoaxial joints and 21.43% in post bone graft area at 3 months postoperatively, and a significant difference was observed (p = 0.022). Besides, the cervical sagittal alignment in all patients was well maintained in the last follow-up compared to preoperatively. Importantly, a complete bony fusion in the atlantoaxial joints was observed in all patients. Moreover, the JOA, NDI, and VAS scores had improved significantly at the last follow-up. CONCLUSION: Bone autografting of the bilateral atlantoaxial joints is a safe and effective technique to increase bone fusion rates, shorten bone fusion time, and reduce complication rates when the cable-dragged reduction and cantilever beam internal fixation approach is used. Therefore, it is a cost-effective surgical procedure for treating patients with atlantoaxial dislocation or instability.

Anatomy of the craniocervical junction - A review.

An in-depth understanding of the anatomy of the craniocervical junction (CCJ) is indispensable in skull base neurosurgery. In this paper, we discuss the osteology of the occipital bone, the atlas (C1) and axis (C2), the ligaments and the muscle anatomy of the CCJ region and their relationships with the vertebral artery. We will also discuss the trajectory of the vertebral artery and review the anatomy of the jugular foramen and lower cranial nerves (IX to XII). The most important surgical approaches to the CCJ, including the far lateral approach, the anterolateral approach of Bernard George and the endoscopic endonasal approach, will be discussed to review the surgical anatomy.

Craniocervical fusion in the paediatric population - case series of 21 patients.

PURPOSE: Craniocervical junction instability in a paediatric population presents a formidable challenge to the neurosurgeon. With a variety of anatomical variations, diminutive bony and ligamentous structures, possible superimposed syndromic abnormalities, the craniocervical junction is a technically challenging region to operate within. We aimed to review our series of patients to identify the common pathologies necessitating craniocervical fusion along with the use of intraoperative image guidance. METHODS: We performed a retrospective review of twenty-one patients, with a mean age of 8.1 years, undergoing craniocervical fixations, involving either occipitocervical fusion or atlantoaxial fixation, from a single institution over a twelve-year period. The presentation, preoperative investigations, indication for surgery, surgical procedure, use of intraoperative navigation and clinical and radiological results were examined. RESULTS: Twelve patients underwent primary C1-2 fixation, with the remaining 9 undergoing occipitocervical fusion. Five patients underwent surgery for traumatic instability, seven for os odontoideum, six for congenital anomalies and three for post-infectious instability. Follow up for 20 patients averaged 34 months (range 2-93 months). Five patients had Trisomy 21 and all underwent C1-2 fixation. Frameless stereotactic image guidance was utilised in five patients. No patients suffered immediate complications resulting from craniocervical junction fusion. Patients were observed to either neurologically improve (n = 6), or arrest their neurological deterioration following surgical intervention (n = 14), with concomitant radiological evidence of osseous fusion at follow up in 18 of 20 patients (90%). Two patients (10%) had radiological evidence of screw lucency, but neither required intervention because of being asymptomatic. CONCLUSIONS: Craniocervical fixation in a paediatric population is a viable and safe treatment modality for the management of disorders requiring stabilisation at the craniocervical junction. Our experience of utilising frameless stereotaxy in the setting of grossly distorted anatomical landmarks is also reported.

Retrospective analysis of atlantoaxial rotatory fixation describing age distribution and gender ratio in children and adolescents: A preliminary report.

INTRODUCTION: Atlantoaxial rotatory fixation (AARF) in children presents with an acute onset of neck pain. Almost all cases heal within a few days of onset and are treated conservatively. Because few cases of AARF have been reported, the age distribution or gender ratio of AARF in the child population have not been described enough. In Japan, the social insurance system covers all citizens. Thus, we used insurance claims data to investigate the features of AARF. The aim of this study is to examine the age distribution, compare gender ratio and determine the recurrence proportion of AARF. METHODS: We used the JMDC database to search for claims data submitted between January 2005 and June 2017 for cases of AARF in patients aged <20 years. RESULTS: We identified 1949 patients with AARF, of which 1102 (56.5%) were male. The mean age was 98.3 ± 42.2 months and 91.6 ± 38.4 months in males and females, respectively, and males with AARF were significantly older at onset than females with AARF (p < 0.001). In both sexes, the highest frequency of AARF occurred when the patient was 6 years old. There were 121 (6.2%) cases of recurrent AARF (male: 61, 5.5%; female: 60, 7.1%), but the age differences between the sexes in these cases were not statistically significant. CONCLUSIONS: This is the first report to describe the characteristics of the study population of AARF. Males were more likely to suffer from AARF than females. Furthermore, age (in months) at AARF onset was significantly higher in males than in females. Recurrence rate was not significant in both sexes.

C1-C2 intraarticular distraction with anterior cervical cages for basilar invagination realignment: Operative technique nuances and review of literature.

Neurosurgical management of basilar invagination (BI) has traditionally been aimed at direct cervicomedullary decompression through transoral dens resection or suboccipital decompression with supplemental instrumented fixation. Dr. Goel introduced chronic atlantoaxial dislocation (AAD) as the etiology in most cases of BI and described a technique for distracting the C1-C2 joint with interfacet spacers to achieve reduction and anatomic realignment. We present our modification to Goel's surgical technique, in which we utilize anterior cervical discectomy (ACD) cages as C1-C2 interfacet implants. A young adult male presented to our institution with BI, cervicomedullary compression, occipitalization of C1, and Chiari 1 malformation. There was AAD of C1 over the C2 lateral masses. This reduced some with preoperative traction. He underwent successful C1-C2 interfacet joint reduction and arthrodesis with anterior cervical discectomy (ACD) cages and concomittant occiput to C2 instrumented fusion. BI can be effectively treated through reduction of AAD and by utilizing ACD cages as interfacet spacers.

Reliability and head-loading effects in imaging diagnosis of vertical subluxation in patients with rheumatoid arthritis.

OBJECTIVES: The purpose of this study was to examine the reproducibility of vertical subluxation (VS) parameters using X-ray, computed tomography (CT), and tomosynthesis (TS) while comparing the head-loading effects. METHODS: The VS parameters of 26 patients (retrospective review) were evaluated. Using the intra-class correlation coefficient, we statistically examined the intra-rater and inter-rater reliabilities of the parameters. Head-loaded and -unloaded imagings were compared using a Wilcoxon signed-rank test. RESULTS: The intra-rater reliability of TS and CT showed intra-class correlation coefficients of ≥0.8 (X-ray range: 0.6-0.8), with similar results for the inter-rater reliabilities. Furthermore, in head-loading imaging, the TS had significantly higher VS scores than that of CT (P < .05). CONCLUSIONS: In comparison with the X-ray, TS and CT were more accurate and reproducible. In terms of head loading, the VS values for TS were worse than those for CT, indicating that TS was more effective than CT in diagnosing VS.