Skull base chordomas are rare, locally osseo-destructive lesions that present unique surgical challenges due to their involvement of critical neurovascular and bony structures at the craniovertebral junction (CVJ). Radical cytoreductive surgery improves survival but also carries significant morbidity, including the potential for occipitocervical (OC) destabilization requiring instrumented fusion. The published experience on OC fusion after CVJ chordoma resection is limited, and the anatomical predictors of OC instability in this context remain unclear.
PubMed and Embase were systematically searched according to the PRISMA guidelines for studies describing skull base chordoma resection and OC fusion. The search strategy was predefined in the authors\' PROSPERO protocol (CRD42024496158).
The systematic review identified 11 surgical case series describing 209 skull base chordoma patients and 116 (55.5%) who underwent OC instrumented fusion. Most patients underwent lateral approaches (n = 82) for chordoma resection, followed by midline (n = 48) and combined (n = 6) approaches. OC fusion was most often performed as a second-stage procedure (n = 53), followed by single-stage resection and fusion (n = 38). The degree of occipital condyle resection associated with OC fusion was described in 9 studies: total unilateral condylectomy reliably predicted OC fusion regardless of surgical approach. After lateral transcranial approaches, 4 studies cited at least 50%-70% unilateral condylectomy as necessitating OC fusion. After midline approaches-most frequently the endoscopic endonasal approach (EEA)-at least 75% unilateral condylectomy (or 50% bilateral condylectomy) led to OC fusion. Additionally, resection of the medial atlantoaxial joint elements (the C1 anterior arch and tip of the dens), usually via EEA, reliably necessitated OC fusion. Two illustrative cases are subsequently presented, further exemplifying how the extent of CVJ bony elements removed via EEA to achieve complete chordoma resection predicts the need for OC fusion.
Unilateral total condylectomy, 50% bilateral condylectomy, and resection of the medial atlantoaxial joint elements were the most frequently described independent predictors of OC fusion in skull base chordoma resection. Additionally, consistent with the occipital condyle harboring a significantly thicker joint capsule at its posterolateral aspect, an anterior midline approach seems to tolerate a greater degree of condylar resection (75%) than a lateral transcranial approach (50%-70%) prior to generating OC instability.

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.

The cranio-vertebral junction (CVJ) was formerly considered a surgical \"no man\'s land\" due to its complex anatomical and biomechanical features. Surgical approaches and hardware instrumentation have had to be tailored in order to achieve successful outcomes. Nowadays, thanks to the ongoing development of new technologies and surgical techniques, CVJ surgery has come to be widely performed in many spine centers. Accordingly, there is a drive to explore novel solutions and technological nuances that make CVJ surgery safer, faster, and more precise. Improved outcome in CVJ surgery has been achieved thanks to increased safety allowing for reduction in complication rates. The Authors present the latest technological advancements in CVJ surgery in terms of imaging, biomaterials, navigation, robotics, customized implants, 3D-printed technology, video-assisted approaches and neuromonitoring.

The craniovertebral junction (CVJ) is a complex transition area between the skull and cervical spine. Pathologies such as chordoma, chondrosarcoma and aneurysmal bone cysts may be encountered in this anatomical area and may predispose individuals to joint instability. An adequate clinical and radiological assessment is mandatory to predict any postoperative instability and the need for fixation. There is no common consensus on the need for, timing and setting of craniovertebral fixation techniques after a craniovertebral oncological surgery. The aim of the present review is to summarize the anatomy, biomechanics and pathology of the craniovertebral junction and to describe the available surgical approaches to and considerations of joint instability after craniovertebral tumor resections. Although a one-size-fits-all approach cannot encompass the extremely challenging pathologies encountered in the CVJ area, including the possible mechanical instability that is a consequence of oncological resections, the optimal surgical strategy (anterior vs posterior vs posterolateral) tailored to the patient\'s needs can be assessed preoperatively in many instances. Preserving the intrinsic and extrinsic ligaments, principally the transverse ligament, and the bony structures, namely the C1 anterior arch and occipital condyle, ensures spinal stability in most of the cases. Conversely, in situations that require the removal of those structures, or in cases where they are disrupted by the tumor, a thorough clinical and radiological assessment is needed to timely detect any instability and to plan a surgical stabilization procedure. We hope that this review will help shed light on the current evidence and pave the way for future studies on this topic.

This paper aimed to better describe the anatomy of the superficial anterior atlanto-occipital ligament of the craniocervical junction and discuss this ligament\'s potential function and clinical implications. A broad literature review on the anatomical features and findings of the superficial anterior atlanto-occipital ligament was performed. The superficial anterior atlanto-occipital ligament is located anterior to the anterior atlanto-occipital membrane. However, the physiological role of the superficial anterior atlanto-occipital ligament is still unclear due to a lack of anatomical and biomechanical studies although one study has suggested that this ligament is a secondary stabilizer of the craniocervical junction. Further studies are needed to clarify the function and anatomy of the superficial anterior atlanto-occipital ligament.

Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes.
A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis.
Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from -3.26 mm to -6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41).
The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

Although historically considered fatal, with the advent of improved pre-hospital care, traumatic dislocation of the craniovertebral junction (CVJ) has been increasing in prevalence in neurosurgical centers. As more survivors are reported each year, a timely review with meta-analysis of their management seems necessary. PRISMA checklist was followed step by step. PubMed and Web of Science databases were searched using words \"craniovertebral junction dislocation\" and their corresponding synonyms. Study eligibility criteria included research studies from 2015 onwards that delineated adult and pediatric patients with confirmed post-traumatic atlantooccipital dislocation (AOD) or atlantoaxial dislocation (AAD) who survived until proper treatment. Of 1475 initial records, 46 articles met eligibility criteria with a total of 141 patients with traumatic CVJ dislocation. Of the patients, 90 were male (63.8%). Mean age of the cohort was 33.3 years (range 1-99 years). Trauma that most often led to this injury was road traffic accident (70.9%) followed by falls (24.6%). The majority of authors support posterior instrumentation of C1-C2 (45.2%) especially by means of Goel-Harms method. At mean follow-up of 15.4 months (range 0.5-60 months), 27.2% of treated patients remained neurologically intact. Of initially symptomatic, 59% improved, 37% were stable, and 4% deteriorated. Instrumenting the occiput in cases of pure AAD was associated with lower chance of neurological improvement in chi-square test (p = 0.0013) as well as in multiple linear regression (β = - 0.3; p = 0.023). The Goel-Harms C1-C2 fusion is currently the most frequently employed treatment. Many survivors remain with no deficits or improve, rarely deteriorate. Involving the occiput in stabilization in cases of AAD without AOD might be related with worse neurological prognosis.

Nonmissile penetrating injuries to the craniocervical junction caused by a glass fragment are rare, and a standard management strategy has not been established.
A 75-year-old Japanese man was brought into our emergency department after receiving a left retroauricular stab wound by broken glass fragments. After spinal immobilization, a computed tomography (CT) scan revealed glass fragments penetrating at the right craniocervical junction to the interatlantooccipital subarachnoid space. CT angiography showed that both vertebral arteries were not injured. Magnetic resonance imaging demonstrated that the glass fragments did not penetrate the cervical cord or medulla oblongata. These glass fragments were removed via a midline incision from the external occipital protuberance to the C7 and with laminectomy without suboccipital craniectomy. Five of the glass fragments were found and removed in total. The dural defect was patched with a free fascia autograft. His postoperative course was uneventful. Postoperative CT angiography showed that both vertebral arteries were intact and the glass fragments had been removed completely.
CT graphical diagnosis is useful for the management of penetrating craniocervical junction trauma, and it should be considered in the evaluation of patients who have suffered craniocervical penetrating injury even in the absence of major wounds or bleeding. Spinal immobilization of patients with craniocervical penetrating injuries is crucial to avoid not only secondary neurologic damage but also secondary critical vascular damage. Incomplete or inadequate assessment of craniocervical stab wounds results in unexpected hazards that are preventable.

Calcifying pseudoneoplasms of the neuroaxis (CAPNON) are rare, slow-growing, fibro-osseous lesions that can occur throughout the entire neuroaxis with few reported cases. We describe a case of craniocervical CAPNON that was treated by our unit and review the available literature. CAPNON are rare benign lesions occurring throughout the neuroaxis. Although rare, these lesions should be considered in cases of histopathological and radiology features of calcified lesions in both brain and spine. Despite a paucity of reports complete safe surgical resection should remain the goal to obtain the best clinical outcomes.

OBJECTIVE: Traumatic tectorial membrane injuries have different radiologic presentations in adult versus pediatric patients. The purpose of this study was to identify and classify the different types of tectorial membrane injuries that occur in the adult and pediatric populations.
METHODS: Patients who suffered tectorial membrane injury were identified retrospectively using the keywords \'tectorial membrane,\" \"craniocervical ligament tear/injury,\" and \"atlanto-occipital dissociation\" included in radiology reports between 2012 and 2018 using Nuance mPower software. All relevant imaging studies were reviewed by two certificates of additional qualification-certified neuroradiologists. Detailed descriptions of injuries were recorded along with any relevant additional findings, including clinical history.
RESULTS: Ten adults and six pediatric patients were identified with acute traumatic injuries of the tectorial membrane. Ninety percent of the adult patients sustained complete disruptions inferior to the clivus, or subclival, with 22% of tears at the level of the basion and 78% at the level of the odontoid tip. In contrast, 83% of pediatric patients suffered a stripping injury of the tectorial membrane located posterior to the clivus, or retroclival. Stretch injuries of the tectorial membrane were identified in 10% of adults and 17% of pediatric patients. The juvenile-type injury, which causes retroclival epidural hematoma, was determined to preferentially occur in patients less than or equal to 14 years of age with a high level of statistical significance (p value = 0.0014).
CONCLUSIONS: A classification system for tectorial membrane injuries is proposed based on this data: type 1-retroclival stripping injury (more common in pediatric patients); type 2a-subclival disruption at the basion and type 2b-subclival disruption at the odontoid (both more common in adult patients); and type 3-thinning of the tectorial membrane.