Search

OBJECTIVE Craniocervical junction (CCJ) chordomas are a neurosurgical challenge because of their deep localization, lateral extension, bone destruction, and tight relationship with the vertebral artery and lower cranial nerves. In this study, the authors present their surgical experience with the endoscope-assisted far-lateral transcondylar approach (EA-FLTA) for the treatment of CCJ chordomas, highlighting the advantages of this corridor and the integration of the endoscope to reach the anterior aspect and contralateral side of the CCJ and the possibility of performing occipitocervical fusion (OCF) during the same stage of surgery. METHODS Nine consecutive cases of CCJ chordomas treated with the EA-FLTA between 2013 and 2020 were retrospectively reviewed. Preoperative characteristics, surgical technique, postoperative results, and clinical outcome were analyzed. A cadaveric dissection was also performed to clarify the anatomical landmarks. RESULTS The male/female ratio was 1.25, and the median age was 36 years (range 14–53 years). In 6 patients (66.7%), the lesion showed a bilateral extension, and 7 patients (77.8%) had an intradural extension. The vertebral artery was encased in 5 patients. Gross-total resection was achieved in 5 patients (55.6%), near-total resection in 3 (33.3%), and subtotal resection 1 (11.1%). In 5 cases, the OCF was performed in the same stage after tumor removal. Neither approach-related complications nor complications related to tumor resection occurred. During follow-up (median 18 months, range 5–48 months), 1 patient, who had already undergone treatment and radiotherapy at another institution and had an aggressive tumor (Ki-67 index of 20%), showed tumor recurrence at 12 months. CONCLUSIONS The EA-FLTA provides a safe and effective corridor to resect extensive and complex CCJ chordomas, allowing the surgeon to reach the anterior, lateral, and posterior portions of the tumor, and to treat CCJ instability in a single stage.

Occipital condylar avulsion fractures are considered potentially unstable, associated with craniocervical dissociation spectrum injuries, and thought to carry a relatively high mortality rate based on the current literature. The purpose of this study was to identify patient with acute, occipital condylar avulsion fractures and evaluate for the incidence of concomitant cervical osteoligamentous trauma and craniocervical dissociation spectrum injury on cervical spine CT and MRI. Patients who suffered an inferomedial occipital condylar avulsion fracture were identified retrospectively using Nuance mPower software. Cervical spine CT and MRI reports performed within 48 h for this patient cohort were then reviewed by two CAQ certified neuroradiologists. Confirmation of an occipital condylar avulsion fracture was recorded along with any concomitant craniocervical junction injury. Relevant clinical history, including management and outcomes, was recorded for each patient. Thirty-four patients were identified with an inferomedial fracture of the occipital condyle. Of the 85% of patients who underwent cervical MRI, all but one patient demonstrated a ‘negative’ MRI without major craniocervical junction ligamentous injury. These patients were treated conservatively with external bracing without persistent neurologic deficits upon 4-month follow-up. Inferomedial fractures of the occipital condyle are currently classified as potentially unstable fractures based on the Anderson classification system. Our data suggest that an isolated occipital condylar avulsion fracture without an additional C1–C2 fracture or widening of the atlanto-occipital joint space is likely a stable injury that can be treated conservatively with excellent clinical outcomes.

Background The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically irrelevant finding. No studies, however, describe its prevalence or whether it is accompanied by other persistent fontanelles (PFs). Although Chihuahuas are predisposed to Chiari-like malformation (CM) and syringomyelia (SM), it is unknown whether PFs occur more commonly in dogs with clinical signs that are caused by CM or SM. Hypothesis/objectives To describe the number and location of PFs at cranial sutures (CSs) and to compare the occurrence of these PFs in dogs with and without CM/SM-related clinical signs. We hypothesized that PFs also occur commonly at lateral and caudal cranial surfaces, affect a higher number of CSs, and are larger in dogs with CM/SM-related clinical signs. Animals Fifty client-owned Chihuahuas with or without CM/SM-related clinical signs. Results Of the 50 dogs evaluated, 46 (92%) had either 1 or several PFs. The mean ± SD number of PFs was 2.8 ± 3.0 (range, 0-13). A total of 138 PFs occupied 118 CSs with 57 (48%) located dorsally, 44 (37%) caudally, and 17 (14%) laterally. The number of CSs affected by PFs was significantly higher (P ≤ .001) and total PF area was significantly larger (P = .003) in dogs with CM/SM-related clinical signs. Conclusions and clinical importance Persistent fontanelles are very common in this group of Chihuahuas and appear at dorsal, lateral, and caudal cranial surfaces. They are more numerous and larger in Chihuahuas with CM/SM-related clinical signs.

Background Persistent fontanelles (PFs) are, in Chihuahuas, almost ubiquitous. Furthermore, Chihuahuas are predisposed to other craniomorphological abnormalities, including syringomyelia (SM), ventriculomegaly, and craniocervical junction (CCJ) overcrowding resulting in neural tissue deviation. It is, however, undetermined if PFs are more common in dogs with these structural abnormalities, and their etiology is unknown. Hypothesis/objectives Persistent fontanelles are more numerous and larger in Chihuahuas with low body weight, older age, SM, dilated fourth ventricle, ventriculomegaly, and CCJ overcrowding. Animals Fifty client-owned Chihuahuas. Methods Cross-sectional study evaluating the association of both the number of cranial sutures affected by PFs (NAS) and total fontanelle area (TFA), based on computed tomography with SM, fourth ventricle dilatation, lateral ventricle volume, and extent of neural tissue compression at the CCJ based on magnetic resonance images. Results The NASs was higher and TFA larger in dogs with low body weight (NAS: P = .007; 95% confidence interval [CI] = 0.384-0.861; TFA: P = .002; 95% CI = -1.91 to -0.478), larger lateral ventricles (NAS: P ≤ .001; 95% CI = 1.04-1.15; TFA: P ≤ .001; 95% CI = 0.099-0.363), and more severe neural tissue compression at the CCJ (NAS: P ≤ .001; 95% CI = 1.26-2.06; TFA: P = .03; 95% CI = 0.066-1.13). Similarly, dogs with SM (NAS: P = .004; 95% CI = 1.26-3.32; TFA: mean ± SD, 130 ± 217 mm2 ; P = .05) had higher NAS and larger TFA than did dogs without SM (43.7 ± 61.0 mm2 ). Age was not associated with NAS (P = .81; 95% CI = 0.989-1.01) or TFA (P = .33; 95% CI = -0.269 to 0.092). Conclusions and clinical importance Persistent fontanelles are associated with small size, SM, ventriculomegaly, and CCJ overcrowding.

Duplication of the odontoid process remains a rare developmental pathology that is underrepresented in the current literature. As the pivot point for the craniovertebral junction, the odontoid process is vital for the integrity of the atlanto-axial joint and the ability of the head and cervical spine to rotate correctly. The pathogenesis being incompletely understood, it has been proposed that odontoid process duplication involves faulty sclerotome migration and disruption of the axis ossification center. Patients presenting with this pathology usually have associated structural abnormalities. A detailed anatomical and embryological understanding of the odontoid process is necessary for successful management and treatment of patients presenting with odontoid process duplication. We present a rare case of a patient with a duplicated odontoid process in association with C2–C3 fusion, incomplete anterior arch of C1, variant inferior bony process of the transverse process of C1, and enlarged right jugular foramen.

Background: To assess changes of the craniocervical junction (CCJ), computed tomography (CT) is considered the reference standard. Recent advances in bone depiction on magnetic resonance imaging (MRI) enable high‐quality visualization of osseous structures. Consequently, MRI may serve as an alternative to CT, without the use of ionizing radiation. Purpose: To compare two MRI sequences optimized for bone visualization to the CT reference standard in the assessment of the osseous CCJ. Study Type: Prospective. Population/Subjects: Twenty‐seven decedents and five healthy volunteers. Field Strength/Sequence: 3T/ultrashort‐echo time gradient echo (UTE) and optimized 3D‐multiecho in‐phase gradient echo sequences (FRACTURE). Assessment: All decedents were scanned with both MRI sequences and CT. Three observers rated degeneration to obtain a score for the upper (atlanto‐dental and left/right atlanto‐occipital joint) and for the lower part of the CCJ (left and right atlanto‐axial joint). Two reader rated the following quantitative parameters: basion‐axial‐interval, atlanto‐dental‐interval, atlanto‐occipital‐interval, Powers‐ratio, and signal/contrast‐to‐noise‐ratio. As a proof of concept, five healthy volunteers were scanned with both MRI sequences. Statistical Tests: Degeneration was assessed on a Likert scale by three independent observers. Interrater and intermodality reliability were calculated using an intraclass correlation coefficient. To compare distance measurements between examination methods, a Friedman test, between‐degenerative ratings, and a Kruskal–Wallis test were performed. Results: Degenerative ratings of the CCJ between MRI sequences and CT showed a good interrater and intermodality agreement. MRI sequences tended to underestimate the degree of degeneration compared to CT, and this became more marked with increasing degeneration severity. There were no significant relationships between distance measurements and the degree of degeneration (PCT = 0.62, PUTE = 0.64, PFRACTURE = 0.67). The in vivo examination proved the feasibility of both MRI methods in a clinical setting. Data Conclusion: Quantitative and qualitative ratings on MR images were comparable to CT images; thus, MRI may be a valid alternative to CT assessing the CCJ. Level of Evidence: 1. Technical Efficacy Stage: 3.

Neurenteric cysts are rare congenital lesions of benign nature that can be encountered at any level of the neuraxis, starting from the cranium down to coccyx. Rewarding outcome can be achieved with early diagnosis and complete removal of these benign lesions. Here, we report a case of a huge neurenteric cyst in an 11-year-old boy at the ventral craniocervical junction, a rarely reported entity with literature review. In this article, we focus on the clinical presentation, pathogenesis, radiological findings, surgery, and surgical outcome of this benign lesion, as we succeeded to have gratifying result following surgery in our instance.

Background Long-term stabilization of the cervical spine after extensive multilevel tumor resection is difficult to achieve. The current standard approach of instrumentation combined with allograft or nonvascularized autograft is limited in settings of increased risk of nonunion or delayed union (i.e., prior radiation therapy or poorly vascularized wound beds). Case Description We report the first time to our knowledge that a vascularized fibular free flap has been used to reconstruct the cervical column across 5 vertebral levels, from the craniocervical junction to the lower cervical spine. We describe a transoral approach to the area and compare this method with other reconstructive options. Conclusions Vascularized bone grafting is a viable alternative to achieve lasting stability because of hastened fusion time, limited reliance on osseous remodeling, and incorporation into the axial skeleton with strut strength.

Horizontal fractures of the atlas are uncommon fractures associated with instability of the craniocervical junction. Most commonly associated with high-speed motor vehicle accidents, these fractures need to be identified and treated appropriately. Due to its relatively benign presentation on bony imaging, magnetic resonance imaging to look for ligamentous instability is important. We present two such cases which were managed by occipitocervical fusion at our institute.

To assess the accuracy of craniocervical measurements for identifying craniocervical injuries and the frequency of subjective findings of craniocervical injuries on CT in pediatric patients. Case-controlled retrospective review of patients ≤ 16 years old with craniocervical junction injuries. Receiver operator curves were created for common craniocervical measurements on CT comparing patients with complete and partial craniocervical injuries to uninjured cohort. Frequency of subjective CT findings of craniocervical injury was assessed in the injured cohort. For complete disruption injuries (CD) (n = 27), C1–C2 distance (AUC = 0.90, 95%CI = 0.83–0.97), atlanto-occipital distance (AUC = 0.95–0.98, 95%CI = 0.90–1.00), and basion-dens distance (AUC = 0.90, 95%CI = 0.82–0.98) had excellent accuracy diagnosing injury. Powers ratio (AUC = 0.85, 95%CI = 0.76–0.94) had good, basion-posterior axial line (AUC = 0.74, 95%CI = 0.61–0.86) fair, and atlanto-dental distance (AUC = 0.69, 95%CI = 0.57–0.82) poor accuracy. For partial disruption injuries (PD) (n = 21), basion-dens distance (AUC = 0.75, 95%CI = 0.62–0.88) had fair accuracy diagnosing injury. Powers ratio (AUC = 0.63, 95%CI = 0.47–0.79), C1–C2 distance (AUC = 0.60, 95%CI = 0.45–0.75), atlanto-dental distance (AUC = 0.55, 95%CI = 0.39 = 0.71), atlanto-occipital distance (AUC = 0.63–0.65, 95%CI = 0.47–0.81), and basion-posterior axial line (AUC = 0.60, 95%CI = 0.44–0.76) all had poor accuracy. Eighty-one percent (n = 22) of CD and 38% (n = 8) of PD patients had non-concentric atlanto-occipital joints. One hundred percent of CD patients had ≥ 1 soft tissue finding and eighty-one percent (n = 22) had ≥ 2 findings. Seventy-three percent (n = 16) of PD patients had ≥ 1 soft tissue finding. Eighty-six percent (n = 18) of PD patients had non-concentric atlanto-occipital joints and/or soft tissue findings. Craniocervical measurements have poor accuracy for identifying craniocervical injuries in pediatric patients with incomplete craniocervical ligament disruption. Subjective findings of craniocervical injury are frequently present on CT in pediatric patients and can help increase sensitivity for identifying injury.

Study Design: In vitro biomechanical study. Objective: The strength of the alar ligament has been described inconsistently, possibly because of the nonphysiological biomechanical testing models, and the inability to test the ligament with both attachments simultaneously. The purpose of this biomechanical model was to reevaluate the alar ligament’s tensile strength with both bony attachments, while also keeping the transverse ligament intact, all in a more physiological biomechanical model that mimics the mechanism of traumatic injury closely. Methods: Eleven fresh-frozen occipito-atlanto-axial (C0-C1-C2) specimens were harvested from individuals whose mean age at death was 77.4 years (range 46-97 years). Only the alar and transverse ligaments were preserved, and the bony C0-C1-C2 complex was left intact. Axial tension was exerted on the dens to displace it posteriorly, while the occipito-axial complex was fixed anteriorly. A device that applies controlled increasing force was used to test the tensile strength (M2-200, Mark-10 Corporation). Results: The mean force required for the alar ligament to fail was 394 ± 52 N (range 317-503 N). However, both the right and left alar ligaments ruptured simultaneously in 10 specimens. The ligament failed most often at the dens (n = 10), followed by occipital condyle rupture (n = 1). The transverse ligament remained intact in all specimens. Conclusions: When both the right and left alar ligament were included, the total alar ligament failure occurred at an average force of 394 N. The alar ligament failed before the transverse ligament.

Care should be taken regarding surrounding anatomic structures during access to deep-seated extracranial schwannomas in the craniocervical junction (CCJ). Herein, we present surgical tips for extracranial schwannomas in the CCJ using the anterolateral approach. A retrospective review was performed of 3 cases of surgical treatment of extracranial schwannomas in the CCJ by the anterolateral approach, which is a presternomastoid retrojugular route to the CCJ. The combination of neck rotation and reflection of the sternocleidomastoid muscle presented a sufficient, shallow surgical field for the CCJ. We could identify tumors along the accessory nerves and internal jugular veins, and had sufficient rostrocaudal working space to resect the tumors. Two cases were enucleated total resection and 1 was subtotal resection. Two patients experienced transient postoperative vocal cord partial paralysis and 1 had transient dysphagia. These neurological complications improved within 1 month. The anterolateral approach can provide a shallow and sufficient rostral and caudal surgical window.

The embryonic occipital bone and odontoid process of the axis are attached and connected by the notochord, but become separated in later development and growth. With special attention to the process of separation, we examined sagittal sections of the craniocervical junction in 18 human fetuses at 8-16 weeks and 22 fetuses at 31-37 weeks. At 8-9 weeks, the anterior arch of atlas was always seen overriding the occipital basal part. The odontoid process was close to the occipital with or without a transient joint cavity until 16 weeks. Near term, the top of the odontoid process was usually higher than the anterior arch, but the former was sometimes (7 of 22) at a level almost equal to or lower than the latter. The apical ligament was evident in a few specimens (5 of 22). A distance between the occipital basion and odontoid process was sometimes less than 1.5 mm (8 of 22) or less than half the thickness of the arch (10 of 22). A transient joint cavity between the basion and odontoid process was often (10 of 22). In three fetuses near term, the atlanto-occipital joint cavity was continuous with the median atlanto-axial joint cavity, and the anterior arch was overriding the occipital basal part. Therefore, rather than stage or age, individual differences were evident in the topographical relationship between the three bony elements at the craniocervical junction. An understanding of the embryology and normal development will aid in the correct interpretation of radiologic images of the pediatric cervical spine.

SUMMARY INTRODUCTION Retro-odontoid pseudotumor (ROP) is a rare disease that affects the atlantoaxial joint and, in general, is associated with local biomechanical alterations that may or may not cause instability. METHODS Descriptive study of the literature available in databases MEDLINE/PubMed, LILACS, and Scopus. The research was conducted in April 2019. DISCUSSION ROP is, possibly, a syndromic designation that encompasses a significant variety of diseases of the atlantoaxial joint. There are different pathophysiological mechanisms implicated in its genesis. The patients, almost in their entirety, present with severe myelopathy, and most of them are treated surgically, with the posterior decompression being the most commonly used method, with or without arthrodesis. Evolution is usually favorable. CONCLUSION The ROP is still poorly recognized as a differential diagnosis between the diseases of the cranial-cervical junction. The information available in the literature analyzed was based mainly on the study of reports or case series; therefore, it is insufficient to define conducts with a high level of scientific evidence.

Background Following head and neck trauma, the involvement of the cranio-cervical junction (CCJ) and its contribution to a patients transition to chronic pain, is poorly understood. The detection of hypermobility in this region is dependent on clinical examination and static imaging modalities such as x-ray, CT and MRI. Sagittal plane hypermobility of the CCJ is evaluated using saggital view, flexion-extension cervical radiographs. Frontal plane hypermobility is typically assessed using lateral bending and open mouth cervical radiographs. Unfortunately there is no established reliability surrounding the use of these measures. This study explores the reliability of radiographic measurements of lateral-bending open-mouth cervical radiographs. Methods Cervical open-mouth lateral-bending X-ray images were collected from 56 different patients between 18 and 60 years of age patients following cervical spine injury. These images were interpreted by two musculoskeletal radiologists and two clinicians (physiatrist and chiropractor), using a standard set of measurements. Measurements included qualitative and quantitative assessments of the amount of asymmetry noted between various osseous landmarks. Reliability statistics were calculated for levels of agreement using kappa coefficients (κ) and Intraclass Correlation Coefficients (ICC) for dichotomous and continuous variables, respectively. Results Reliability (κ) for qualitative assessments were moderate to substantial for asymmetry of neutral C2 spinous position, dens-lateral mass spacing, and “step off” between the lateral borders of the articular pillars of C2 and C1 lateral mass (κ range = .47–.78). ICC values for the quantitative measurements of dens-lateral mass spacing and “step off” between the lateral borders of the C2 articular pillars and C1 lateral mass were moderate to excellent (ICC range = .56–.97). Conclusions The qualitative and quantitative measurements used in this study demonstrated good to excellent inter-examiner reliability. Correlation with clinical findings is necessary to establish the utility of these measurements in clinical practice.

Purpose/Aim: Intradural extramedullary (IDEM) ependymomas are very rare, and IDEM ependymomas with craniospinal disseminated metastasis are exceptionally rare; only 2 preoperative cases have been confirmed, and postoperative cases have not been reported.Case report: We present a case of a 21-year-old female with an IDEM ependymoma of the craniocervical junction who experienced head and neck pain for more than 1 month. Magnetic resonance imaging (MRI) of the cervical spine revealed a large IDEM cystic lesion located in the medulla oblongata and the upper cervical spinal cord. The patient underwent surgery without complications, and the tumor was completely removed. Histopathological examination revealed a diagnosis of aplastic ependymoma, World Health Organization (WHO) grade III. The patient failed to follow-up with radiotherapy for one month after discharge. Nearly three months after surgery, craniospinal disseminated metastasis was found in the patient; subsequently, chemoradiotherapy was administered to prolong the survival time of the patient. Unfortunately, the patient underwent radiotherapy and chemotherapy for only 7 days; then, the patient gave up treatment and died 5 months later.Conclusions: To the best of our knowledge, no other cases of craniocervical junction anaplastic ependymomas with craniospinal disseminated metastasis have been reported in the literature. Total resection does not completely prevent recurrence and metastasis, and MRI of the entire neuraxis and timely postoperative craniospinal radiotherapy are necessary for the treatment of this disease.

Lesions posterior to the odontoid process pose a surgical challenge. Posterolateral approaches to this region are considerably risky for the spinal cord. Transoral approaches are limited in terms of exposure and can also carry morbidity. We describe a focused endoscopic endonasal approach (EEA) for removing an osteochondroma located dorsal to the odontoid process. The surgical pearls and pitfalls using stepwise image-guided EEA cadaveric dissections are highlighted defining the importance of various craniocervical junction (CCJ) lines on imaging. EEA to CCJ can be offered, with lower morbidity than other approaches, even for lesions that extend posterior and caudal to the odontoid process. Radiologic predictors of exposure and intraoperative techniques to enhance endoscopic visualization are discussed.

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Posterior fusion of the craniocervical junction (CCJ) has always been challenging in children with rare congenital diseases and malformations. At our institution, the introduction of the translaminar C2 screw technique led to a significant improvement in the quality of treatment. Retrospective analysis of a pediatric cohort at a single institution who underwent CCJ posterior fusion between 2007 and 2018. Patients were divided into group 1 (other posterior fusion techniques, n = 12) and group 2 (translaminar axis screw placement, n = 19). Diagnosis, sex, age at surgery, surgical technique, immobilization, revisions, fusion, reduction, and complications were assessed. Follow-up ranged from 12 to 145 months (mean 50.7). The initial fusion rate detected at 3 months by CT differed significantly (66, 7% in group 1 vs. 100% in group 2, p = 0.018). Full reduction of C1/C2 malalignments was achieved in 41, 6% of group 1 versus 84, 2% of group 2 (p = 0.007). Immobilization was applied in 83, 3% of group 1 versus 26, 3% of group 2 (p = 0.0032). Ten complications were treated conservatively, and 15 events required revision surgery (80% in group 1 vs. 20% in group 2). Eight complications were related to immobilization. The implementation of the translaminar C2 technique resulted in significantly more safety and efficiency regarding pediatric posterior fusion CCJ surgery at our institution, with significantly higher rates of rigid fixation, full reduction, and fusion, and significantly lower rates of complications and immobilization. These slides can be retrieved under Electronic Supplementary Material.

SUMMARY: The aim of this study was to establish safety ranges for the third vertebral artery segment (V3) for craneocervical procedures. Injury to V3 represents a potentially catastrophic complication. Its tortuous path and complex relationship with neighboring structures, increasing the risk. Ten male adult cadavers (20 vertebral arteries) with arterial infiltration of red latex were studied. The length, angles and anatomical measurements were obtained between the selected surgical landmarks and the portions of V3 segment. The horizontal portion has a length of 32.7 ± 3.6 mm with an angulation of 115.1 ± 8.3 degrees. The mean distances of the horizontal portion were: from the midline to the V3 groove of C1 posterior arch (24.7 ± 6.3 mm); from C1 pars interarticularis to the V3 distal loop of V3 (8.9 ± 1.4 mm). The vertical portion has a length 32.5 ± 5.6 mm with an angulation of the proximal loop of 113.6 ± 5.8 degrees. The mean distances between the C2 spinous process to the medial surface of the distal loop (43.8 ± 4.2 mm); from the C1-C2 joint to the V3 vertical portion (9.5 ± 1.5 mm); from C2 pars interarticularis to V3 in the C2 transverse foramen (6.5 ± 3.4 mm); from C2 pars interarticularis to V3 in the C1 transverse foramen (17.5 ± 4.5 mm). We reported four potential sites where V3 can be injured during four different surgical procedures: exposure of the posterior arch of C1, and pars interarticularis of C1 in the horizontal portion and exposure of the C1C2 joint, and placement of C1-C2 transarticular screws one in the vertical portion. We provide measurements of redundancy and safety ranges to reduce the risk of injury to the V3 segment during craniocervical surgical procedures.

Study Design: In vitro biomechanical study. Objective: The transverse ligament is the strongest ligament of the craniocervical junction and plays a critical role in atlanto-axial stability. The goal of this cadaveric study, and the subsequent study (part II), was to reevaluate the force required for the transverse ligament and alar ligament to fail in a more physiological biomechanical model in elderly specimens. Methods: Twelve C1-2 specimens were harvested from fresh-frozen Caucasian cadavers with a mean age at death of 81 years (range 68-89 years). Only the transverse ligament was preserved, and the bony C1-2 complex was left intact. The dens was pulled away from the anterior arch of C1 using a strength test machine that applies controlled increasing force. After testing, the axis was split in half to check for hidden pathologies and osteoporosis. The differences in the failure force between sex and age groups (group 1: 80 years) were compared. Results: The mean force required for the transverse ligament to fail was 236.2 ± 66 N (range 132-326 N). All but 2 specimens had significant osteoporotic loss of trabecular bone. No significant differences between sex and age groups were found. Conclusions: The transverse ligament’s failure in elderly specimens occurred at an average force of 236 N, which was lower than that reported in the previous literature. The ligament’s failure force in younger patients differs and may be similar to the findings published to date.

Intradural spinal lipomas are extremely rare tumors, especially in the craniocervical junction. In a review of the literature, few articles share details on such cases and the best practices for clinical management of this pathology. In the following report, the authors present the case of a 62-year-old man who presented with acute onset dizziness and headache. A CT scan of the cervical spine revealed fatty tissue that was further confirmed by an MRI to be an intradural lipoma at C1-C2 level causing minimal mass effect on spinal cord without myelopathy or hydrocephalus. Therefore, the patient was treated conservatively through an appropriate course of medications with complete resolution of his symptoms.

Stability of the upper cervical spine is highly dependent on ligaments and membranes. These are best appreciated on magnetic resonance imaging (MRI), which can reveal damage to these structures in traumatic, congenital, infectious, and inflammatory conditions. It can also be used to evaluate the spinal cord and spinal canal to assess neurologic compression and cord pathology. Given the potential for devastating neurologic consequences in the setting of upper cervical instability and neural structure compression or injury, MRI is an invaluable tool for evaluation of the craniocervical junction.

Introduction Casamassima-Morton-Nance syndrome (CMNS) is a rare pathology with few reports in the literature. CMNS, Jarcho-Levine syndrome and the Lavy-Moseley syndrome must be considered among the differential diagnoses on those patients with vertebral and costal arch segmentation deficiencies. Objectives We report a case of a 13-year-old girl without a history of consanguinity presenting to the spine clinic with cervicalgia and was found to have altered segmentation of her spine and costal arcs as well as a Chiari type I malformation. Conclusion CMNS is a rare condition with few reported cases in the literature. Due to the associated conditions patients have a short life span. In this report, we present the case of a 13-year-old girl with CMNS, CC junction abnormalities, and Chiari type I malformation.

BACKGROUND Chordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20. Of these pediatric chordomas, those affecting the craniocervical junction (C1–C2) are even more rare; therefore, parameters for surgical management of these pediatric tumors are not well characterized. OBSERVATIONS In this case, a 3-year-old male was found to have a clival chordoma on imaging with extension to the craniocervical junction resulting in spinal cord compression. Endoscopic-assisted transoral transclival approach for clival tumor resection was performed first. As a second stage, the patient underwent a left-sided far lateral craniotomy and cervical laminectomy for resection of the skull base chordoma and instrumented fusion of the occiput to C3. He made excellent improvements in strength and dexterity during rehab and was discharged after 3 weeks. LESSONS In pediatric patients with chordoma with extension to the craniocervical junction and spinal cord compression, decompression with additional occipito-cervical fusion appears to offer a good clinical outcome. Fusion performed as a separate surgery before or at the same time as the initial tumor resection surgery may lead to better outcomes.

Background: Several case reports about spinal cord compression due to hyperostosis at the craniocervical junction are available. However, compression at C1-C2 solely due to ossification of the posterior longitudinal ligament (OPLL) is rare. Case Description: A 50-year-old Asian male, with a history of lumbar spinal canal stenosis, presented with a progressive quadriparesis within 3 months. Imaging showed central OPLL at the C1-C2 level contributing to severe spinal cord compression. The patient improved neurologically after a C1-C2 laminectomy. Conclusion: A patient presented with a progressive Brown-Séquard syndrome due to OPLL at the craniocervical junction (C1-C2 level) and improved following a decompressive laminectomy.

We report a case of craniocervical junction dural arteriovenous fistula (dAVF) presented with myelopathy and normal pressure hydrocephalus, and was treated with hybrid approach of embolization and surgical disconnection. A 68-year-old gentleman presented with 1 year history of unsteady gait and sphincter disturbance. Magnetic resonance imaging (MRI) showed abnormally enlarged and tortuous vessels over right cerebellomedullary cistern. Digital subtraction angiogram (DSA) showed Cognard's type-V dAVF at craniocervical junction. Catheter embolization was performed via external carotid artery and finally surgical disconnection was done with far lateral approach (Fig. 1). Postoperative DSA showed no more arteriovenous shunting (Fig. 2). Clinically the patient improved after a course of rehabilitation. Dural AVF at craniocervical junction is rare and its clinical presentation can be highly variable from subarachnoid hemorrhage to brainstem dysfunction. Identification of the exact fistula site is essential in surgical planning. Surgery is effective and safe to achieve complete obliteration and good clinical outcome.1 2 3 4 5 6 The link to the video can be found at: https://youtu.be/xI48stSlWpY.

OBJECTIVEPrior studies have provided conflicting evidence regarding the contribution of key ligamentous structures to atlantoaxial (AA) joint stability. Many of these studies employed cadaveric techniques that are hampered by the inherent difficulties of testing isolated-injury scenarios. Analysis with validated finite element (FE) models can overcome some of these limitations. In a previous study, the authors completed an FE analysis of 5 subject-specific craniocervical junction (CCJ) models to investigate the biomechanics of the occipitoatlantal joint and identify the ligamentous structures essential for its stability. Here, the authors use these same CCJ FE models to investigate the biomechanics of the AA joint and to identify the ligamentous structures essential for its stability.METHODSFive validated CCJ FE models were used to simulate isolated- and combined ligamentous–injury scenarios of the transverse ligament (TL), tectorial membrane (TM), alar ligament (AL), occipitoatlantal capsular ligament, and AA capsular ligament (AACL). All models were tested with rotational moments (flexion-extension, axial rotation, and lateral bending) and anterior translational loads (C2 constrained with anterior load applied to the occiput) to simulate physiological loading and to assess changes in the atlantodental interval (ADI), a key radiographic indicator of instability.RESULTSIsolated AACL injury significantly increased range of motion (ROM) under rotational moment at the AA joint for flexion, lateral bending, and axial rotation, which increased by means of 28.0% ± 10.2%, 43.2% ± 15.4%, and 159.1% ± 35.1%, respectively (p ≤ 0.05 for all). TL removal simulated under translational loads resulted in a significant increase in displacement at the AA joint by 89.3% ± 36.6% (p < 0.001), increasing the ADI from 2.7 mm to 4.5 mm. An AACL injury combined with an injury to any other ligament resulted in significant increases in ROM at the AA joint, except when combined with injuries to both the TM and the ALs. Similarly, injury to the TL combined with injury to any other CCJ ligament resulted in a significant increase in displacement at the AA joint (significantly increasing ADI) under translational loads.CONCLUSIONSUsing FE modeling techniques, the authors showed a significant reliance of isolated- and combined ligamentous–injury scenarios on the AACLs and TL to restrain motion at the AA joint. Isolated injuries to other structures alone, including the AL and TM, did not result in significant increases in either AA joint ROM or anterior displacement.

Introducción: La invaginación basilar es una malformación compleja de la unión cráneo cervical donde no se ha llegado a consensos de tratamiento y el enforque terapéutico debe individualizarce según el caso y las variantes anatómicas asociadas. En los casos más graves un adecuado enfoque terapéutico pude determinar regresión importante o total de la sintomatología neurológica. Objetivos: El objetivo de esta revisión es llegar al análisis de los algoritmos de tratamiento en base a una comprensión adecuada de la anatomía, variantes anatómicas y conceptualización lo más cercana posible al diagnóstico más correcto para la aplicación del mejor método de tratamiento. Métodos: Se realizó una consulta bibliográfica de los principales trabajos existentes sobre malformaciones de la unión craneocervicales colocando el énfasis en el diagnóstico anatomo morfológico, biomecánica y fisiología y radiológico. Conclusión: La adecuada conceptualización y clasificación de la patología permite tomar como marco referencial para el tratamiento con los algoritmos propuestos por Goel y Sonntag. Sin embargo, cada paciente tiene características individuales y el concepto en sí constituye un continuo de malformaciones que deberán ser resueltas por diferentes vías quirúrgicas, en diferentes tiempos si es necesario y apoyado en las diferentes tecnologías de apoyo actual como neuronavegación y seguimiento neurofisiológico trans operatorio.

Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.

Background The far-lateral approach is a mainstay in gaining access to the ventrolateral craniocervical junction for tumor removal and the treatment of vascular lesions. It has a reportedly high rate of cerebrospinal fluid leak (up to 20%), which can bring devastating consequences, including meningitis, and may require wound revision associated with a longer hospital stay. Objective To describe a closure technique employed to close the access corridor provided by the far-lateral approach and present an illustrative case. Methods The far-lateral closure technique employs dural closure, followed by fat buttress, to alleviate dead space and reduce the likelihood of fluid collection and leakage. Results This technique has been successfully used by the senior author for more than 14 yr, with a rate of cerebrospinal fluid leak of 2.9%. Conclusion This unique approach and closure of the far-lateral craniotomy is a reasonable option for the approach to the ventrolateral craniocervical junction. Skull base surgeons can consider the use of this closure technique to ensure watertight closure.

Imaging of the craniocervical junction (CCJ) after trauma is crucial for injury detection and description. Of all the anatomic measurements of the CCJ, the normal value of atlanto-occipital interval (AOI) is not yet well established in adults. The aim of our study is to determine the normal values of the AOI in Nigerians aged between 21 and 60 years using multidetector computed tomography (MDCT) scan and to determine racial, age, and sex differences.This was a prospective observational study done at Memfys Hospital for Neurosurgery, Enugu, Nigeria, between May 2016 and April 2017. There were 240 subjects (120 men and 120 women) divided into the following age groups: 21-30, 31-40, 41-50, and 51-60 years. Non-Nigerians and those with preexisting CCJ abnormalities were excluded. Normal values were those within the 95% confidence interval. Results were analyzed for possible sex and age variations and compared with the available literature for racial differences. Ethical approval was obtained for this study.The measured normal computed tomography value of the AOI in adult Nigerians was 1.181 ± 0.018 mm. The values in the age groups of 21-30, 31-40, 41-50, and 51-60 years were 1.279 ± 0.038, 1.164 ± 0.033, 1.151 ± 0.029, and 1.133 ± 0.033 mm, respectively (P0.05). The average normal values in men and women were 1.188 ± 0.025 and 1.174 ± 0.026 mm, respectively. The interobserver reliability test using the Cronbach α was 0.938.AOI values in this study were higher than previous MDCT-based values in non-African populations. The AOI decreased linearly with age and did not show any sex difference.

Intradural spinal lipomas are rare in an adult population. They are mostly asymptomatic and usually associated with spinal dysraphism in a pediatric population. We report a rare case of spinal lipoma without dysraphism and with progressing hemiparesis. A 60-year-old woman had incidental lipoma at the craniocervical junction observed for more than 5 years. Recently, she developed right-sided hemiparesis and sensory disturbance. Radiological studies revealed a large lipoma compressing the dorsal medulla and C1–C2 spinal cord. Standard midline suboccipital craniotomy and C1 laminectomy were performed, and the lipoma was removed subtotally. The lipoma showed severe adhesion to the dorsal medulla and C1 spinal cord; therefore, the excision was limited as internal debulking. Her neurological deficit subsided within 6 months after the decompressive surgery. Considering the benign nature of lipoma, internal decompression is a reasonable management for this lesion.

Synovial chondromatosis is an uncommon benign neoplasm that usually affects large appendicular joints and only rarely the spine. There are only a few small series and case reports documenting malignant transformation of synovial chondromatosis into secondary chondrosarcoma, typically within the hip in the setting of recalcitrant disease and multiple recurrences. Chondrosarcoma arising in synovial chondromatosis of the spine is exceedingly rare, with only one previously published case report involving the craniocervical junction. We present a case of chondrosarcoma arising within synovial chondromatosis of the lumbosacral spine, with the diagnosis made at the time of initial presentation. We describe the clinical, imaging, and histopathological findings and review diagnostic criteria for this difficult diagnosis.

Metastases involving the clivus and craniocervical junction (CCJ) are extremely rare. Skull base involvement can result in cranial nerve palsies, while an extensive CCJ involvement can lead to spinal instability. We describe an unusual case of clival and CCJ metastases presenting with VI cranial nerve palsy and neck pain secondary to CCJ instability from metastatic bladder urothelial carcinoma. The patient was first treated with an endoscopic endonasal approach to the clivus for decompression of the VI cranial nerve and then with occipitocervical fixation and fusion to treat CCJ instability. At the 6-month follow-up, the patient experienced complete recovery of VI cranial nerve palsy. To the best of our knowledge, the simultaneous involvement of the clivus and the CCJ due to metastatic bladder carcinoma has never been reported in the literature. Another peculiarity of this case was the presence of both VI cranial nerve deficit and spinal instability. For this reason, the choice of treatment and timing were challenging. In fact, in case of no neurological deficit and spinal stability, palliative chemo- and radiotherapy are usually indicated. In our patient, the presence of progressive diplopia due to VI cranial nerve palsy required an emergent surgical decompression. In this scenario, the extended endoscopic endonasal approach was chosen as a minimally invasive approach to decompress the VI cranial nerve. Posterior occipitocervical stabilization is highly effective in avoiding patient’s neck pain and spinal instability, representing the approach of choice.