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Retrospective case series. The aim of this study was to evaluate the clinical outcomes and effectiveness of using 3D printed implants in upper cervical spine and occipitocervical junction surgery. C2 primary tumor patients who required axial en bloc resection and other patients who required partial bone decompression using customized 3D printed implants or fixation devices for surgery were included. Evaluate the stability and surgical outcomes of 3D printed implants through perioperative and follow-up period. Five tumor patients underwent reconstruction using customized 3D printed artificial vertebral bodies, while another five patients with atlantoaxial joint dislocation underwent reduction and decompression using customized 3D printed internal fixation devices. The postoperative imaging results showed that the 3D printed structures had good immediate stability and had no signs of displacement or subsidence. Follow up showed that all five cases of vertebral body reconstruction had achieved fusion. Only one patient died one month after surgery due to infection and respiratory difficulties. Other patients showed excellent improvement in neurological function in follow up. The use of 3D printed implants in surgery involving the occipitocervical area is a feasible and reliable alternative choice. It is a valuable attempt for complex atlantoaxial dislocation that cannot be treated with conventional instruments. 3D printed implants can improve the safety and accuracy of surgery, provide good immediate stability, have a low incidence of subsidence, fewer related complications during the follow-up period. [ABSTRACT FROM AUTHOR]

Rosai-Dorfman disease (RDD) with craniocervical junction involvement is a rare clinical entity. We present herein a case of a pediatric patient with craniocervical junction RDD which was surgically treated. A 10-year-old female with a history of B-cell acute lymphoblastic leukemia (B-ALL) in remission and RDD presented with frontal migraine headaches. She previously had a right posterior chest wall lesion which was biopsy-proven RDD. She was found on imaging to have a dural-based right craniocervical junction lesion. Given her history of B-ALL, after a multidisciplinary discussion, the decision was made to proceed with resection with possible initiation of cobimetinib or clofarabine. The patient underwent a suboccipital craniotomy, C1 laminectomy, and resection of the dural-based lesion. Gross total resection was achieved, and histopathology confirmed the diagnosis of RDD. She was discharged home on postoperative day 4. No recurrence was seen on follow-up imaging at 3 months. We conducted a systematic literature review examining all cases of pediatric intracranial RDD and all cases of craniocervical junction RDD. This represents, to the best of our knowledge, only the second case of pediatric craniocervical junction RDD. Although RDD is often self-limiting, medical treatment is often considered for intracranial disease, but tissue confirmation is necessary. Surgical resection provides histopathologic diagnosis and can sometimes serve as definitive treatment for a particular lesion. [ABSTRACT FROM AUTHOR]

Background: The management for craniocervical junction dural arteriovenous fistulas (CCJ-DAVFs) remains controversial and clinically challenging. We systemically summarized the clinical and angiographic outcomes of microsurgery, embolization, and conservative management. Methods: Relevant articles were retrieved from PubMed, Scopus, Web of Science, and Cochrane, following PRISMA guidelines. A systematic review and meta-analysis were conducted on the clinical characteristics, management approaches, and clinical and angiographic outcomes. Results: We included 13 articles (166 patients). The weighted mean age was 58.9 years (95%CI: 53.2–64.5), 58.8 years (95%CI: 48.4–69.2), and 63.8 years (95%CI: 60.1–67.5), in microsurgery, embolization, and conservative groups respectively, with an overall male sex predominance (microsurgery [n = 51/77, 66.2%], embolization [n = 44/56, 78.6%], and conservative management [n = 6/8, 75.0%]). Patients were managed with microsurgery (n = 80/172, 46.5%), embolization (n = 79/172, 45.9%), and conservative treatment (n = 13/172, 7.6%). Foramen magnum was the most common location (microsurgery [n = 34/77, 44.2%], embolization [n = 31/56, 55.4%], and conservative treatment [n = 3/8, 37.5%]). Vertebral artery was the primary feeder (microsurgery [n = 58/84, 69.1%], embolization [n = 41/86, 47.6%], and conservative treatment [n = 4/7, 57.1%]). Complete fistula obliteration rates were 74.1% (95%CI:52.3–88.2%) in the microsurgery group and 54.9% (95%CI:30.7–77.0%) in the embolization group. Complications rates were 16.2% (95%CI:6.7–34.5%) in the embolization group, 11.6% (95%CI:3.8–30.4%) in the microsurgery group, and 7.7% (95%CI:1.1–39.1%) in the conservative group. Different rates of good clinical outcomes were observed [microsurgery: 66.4% (95%CI:48.1–80.8%), embolization: 51.9% (95%CI:30.8–72.4%), and conservative: 11.6% (95%CI:4.4–27.4%)]. Conclusions: In patients with CCJ-DAVFs, each management approach has its own merits based on the fistula and patient characteristics. [ABSTRACT FROM AUTHOR]

This paper presents the anatomical and biomechanical aspects of chronic instability of the craniocervical junction (CCJ) with a discussion on clinical diagnostics based on mobility tests and provocative tests related to ligamentous system injuries, as well as radiological criteria for CCJ instability. In addition to the structural instability of the CCJ, the hypothesis of its functional form resulting from cervical proprioceptive system (CPS) damage is discussed. Clinical and neurophysiological studies have shown that functional disorders or organic changes in the CPS cause symptoms similar to those of vestibular system diseases: dizziness, nystagmus, and balance disorders. The underlying cause of the functional form of CCJ instability may be the increased activity of mechanoreceptors, leading to "informational noise" which causes vestibular system disorientation. Due to the disharmony of mutual stimulation and the inhibition of impulses between the centers controlling eye movements, the cerebellum, spinal motoneurons, and the vestibular system, inadequate vestibulospinal and vestibulo-ocular reactions occur, manifesting as postural instability, dizziness, and nystagmus. The hyperactivity of craniocervical mechanoreceptors also leads to disturbances in the reflex regulation of postural muscle tone, manifesting as "general instability". Understanding this form of CCJ instability as a distinct clinical entity is important both diagnostically and therapeutically as it requires different management strategies compared to true instability. Chronic CCJ instability significantly impacts the quality of life (QOL) of affected patients, contributing to chronic pain, psychological distress, and functional impairments. Addressing both structural and functional instability is essential for improving patient outcomes and enhancing their overall QOL. [ABSTRACT FROM AUTHOR]

Our recent studies indicated that a high-riding vertebral artery (HRVA) is a common variant posing a risk of injuring the vessel during C2 instrumentation. However, several different types fit in the current definition of HRVA, which may require a different strategy for C2 screw placement. To classify HRVA and provide a clinical aid for preoperative planning of C2 instrumentation. A secondary goal was to estimate coexistence of each HRVA type with the ipsilateral narrow C2 pedicle (NP). A retrospective observational study involving radiologic measurements of the estimated number of anonymized cervical computed tomography (CT) scans. STROBE checklist was applied. A total of 908 potential screw insertion sites (PSIS) of 454 consecutive cervical CT scans were analyzed. The sample size was estimated using ScalaR SP function in RStudio. Three types of HRVA based on a series of C2 vertebral morphological parameters including the C2 isthmus height (C2IsH) and C2 internal height (C2InH). Also, the prevalences of each HRVA type and coexisting NP based on the C2 pedicle width (C2PW). HRVA was defined as C2IsH of ≤5 mm and/or C2InH of ≤2 mm measured 3 mm lateral to the lateral border of the spinal canal. A narrow pedicle was defined as C2PW of ≤4 mm. Measurements were done using Syn.govia software. Interobserver, intraobserver, and inter-software agreement coefficients for C2IsH, C2InH, and C2PW parameters were adopted from our previous study. K-means cluster analysis was applied. Prevalence of at least 1 HRVA was 24.9% (n=113 subjects) and 16.2% of PSIS (n=147 sites). Based on the measurements and K-means clustering, the following 3 types of HRVA have been distinguished: type 1—isthmic with only C2IsH being reduced and normal C2InH; type 2—internal with only C2InH being reduced and C2IsH within normal limits; type 3—isthmo-internal with both C2IsH and C2InH being reduced. Kruskal-Wallis test followed by unadjusted and Bonferroni-adjusted posthoc multiple comparison analysis detected significant differences across the types. The prevalences of the newly identified types were as follows: 78.2%, 8.8%, and 12.9% for type 1, type 2, and type 3, respectively. 73.9% of type 1 HRVA, 53.8% of type 2 HRVA, and 100% of type 3 HRVA had a concomitant ipsilateral NP. Prediction of the HRVA types by the K-means clustering has been evaluated. Screw placement techniques for each type are proposed and discussed. We present the first classification system for the high-riding vertebral artery distinguishing 3 types based on the large homogenous cohort, which may serve as an adjunct to preoperative planning of C2 instrumentation. External validation of this classification scheme shall determine its further clinical utility. [ABSTRACT FROM AUTHOR]

This study aims to discuss the identification of the C1 nerve root as an effective surgical approach to successfully locate the shunting point of craniocervical junction spinal dural arteriovenous fistula (CCJ-SDAVF) intraoperatively. This study included all patients with CCJ-SDAVF who underwent surgical treatment using the far-lateral transcondylar approach at a single institution from January 2017 to June 2023. Data on patient demographics, clinical and angiographic characteristics of CCJ-SDAVF, surgical details, and treatment outcomes were collected. Follow-up assessments were conducted for all patients until December 31, 2023. The study included a total of 7 patients, comprising 5 men(71.4%) and 2 women (28.6%), with an average age of 57.6 years. Among them, 4 patients (57.1%) developed diffuse subarachnoid hemorrhage(SAH), while 2 patients (28.6%) experienced progressive cervical myelopathy. The shunting points of all CCJ-SDAVFs, which exhibited engorged veins, were identified next to the C1 root. Complete obliteration of CCJ-SDAVFs was successfully achieved in all patients, as confirmed by postoperative angiography one month later. No recurrent CCJ-SDAVFs were observed two years after the operation. Among the patients, 5 (71.4%) experienced good functional recovery, as indicated by an mRS score ranging from 0 to 1, while the remaining 2 patients (28.6%) showed incomplete functional recovery. The surgical interruption of CCJ-SDAVFs is the preferred treatment option, given its high obliteration rate and favorable functional recovery outcomes. We advocate the identification of C1 spinal nerve root as a crucial surgical step to identify the shunting points of CCJ- SDAVFs. [ABSTRACT FROM AUTHOR]

To evaluate the feasibility of a novel method for occipitocervical fixation (OCF) through the endonasal corridor. Thin-cut computed tomography scans were obtained for 5 cadaveric specimens. Image segmentation was used to reconstruct 3D models of each O-C1 joint complex. Using computer-aided design software, plates were custom-designed to span each O-C1 joint, sit flush onto the bony surface, and accommodate screws. The final models were 3D-printed in titanium. For implantation, specimens were held in pin-fixation and registered to neuronavigation. A rigid 0º endoscope was used for endonasal visualization. An inverted U-shaped nasopharyngeal flap was raised to expose the occipital condyles and C1. The plates were introduced and fixed with bone screws. Computed tomography scans were obtained to assess screw accuracy and proximity to critical neurovascular structures. Screw entry points and trajectories were recorded. Endonasal OCF was performed on 5 cadaveric specimens. The mean starting point for occipital condyle screws was 6.17 mm lateral and 5.38 mm rostral to the medial O-C1 joint. Mean axial and sagittal trajectories were 7.98° and 6.71°, respectively. The mean starting point for C1 screws was 16.11 mm lateral to the C1 anterior tubercle and 6.39 mm caudal to the medial O-C1 joint. Mean axial and sagittal trajectories were 10.97° and −9.91°, respectively. Endonasal OCF is technically and anatomically feasible. The application of this technique may allow for same-stage endonasal decompression and fixation, offering a minimally invasive alternative to current methods of fixation and advancing surgeons' ability to treat pathology of the craniovertebral junction. Next steps will focus on biomechanical testing. [ABSTRACT FROM AUTHOR]

Purpose: The topic of osseous variations of the craniocervical junction is a complex morphological and embryological chapter of human anatomy, with a possible impact on neurogical and vascular functionality in this morphological variable region. Material & methods: An until now undescribed anatomical variation of the exoccipital part of the occipital bone has been observed after maceration at the outer skull base of a West-European 68-year-old male body donor. Results: On both sites of the foramen magnum accessory osseous processes were observed that arise from the jugular process and point towards the lateral margin of the foramen magnum. On the left site this process forms a full arc that bridges the condylar fossa completely. Conclusion: The observed osseous bridge over the condylar fossa has not been reported on before and can be explained by the partial persistence of a primordial vertebra between atlas and occipital bone: the Proatlas. The resulting accessory structure may affect due to its topographic conditions the V3-Segment of the vertebral artery and its accompanying nerves, and thus, play a role in diagnosis and therapy of vascular and/or neurological symptoms of head and neck. [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]

Abstract Objective Autologous iliac bone is commonly used as a bone graft material to achieve solid fusion in craniocervical junction (CVJ) surgery. However, the developing iliac bone of children is less than ideal as a bone graft material. The matured rib bone of children presents a potential substitute material for iliac bone. The aim of this study was to evaluate the efficacy of autologous rib grafts for craniocervical junction surgery in children. Methods The outcomes of 10 children with abnormalities of the craniocervical junction who underwent craniocervical junction surgery between January 2020 and December 2022 were retrospectively reviewed. All patients underwent posterior fusion and internal fixation surgery with autologous rib grafts. Pre- and post-operative images were obtained and clinical follow-ups were conducted to evaluate neurological function, pain level, donor site complications, and bone fusion rates. Results All surgeries were successful. During the 8- to 24-month follow-up period, all patients achieved satisfactory clinical results. Computed tomography at 3–6 months confirmed successful bone fusion and regeneration of the rib defect in all patients with no neurological or donor site complications. Conclusion Autologous rib bone is a safe and effective material for bone grafting in craniocervical junction fusion surgery for children that can reduce the risks of donor site complications and increase the amount of bone graft, thereby achieving a higher bone fusion rate.

BACKGROUND/AIM: Craniocervical junction (CCJ) can be involved in inflammatory arthritis. We aimed to define types of CCJ involvement in rheumatoid arthritis (RA), spondyloarthritis (SpA), and psoriatic arthritis (PsA) and compare them with patients without inflammatory arthritides. MATERIALS AND METHODS: In this retrospective analysis, cervical CT or MRIs of patients with RA, SpA, or PsA, taken for any reason between 2010 and 2020, according to ICD-10 codes, were scanned. Demographic data of the patients were recorded. CCJ involvements (atlantoaxial, vertical, or subaxial subluxation, odontoid process involvement) were reevaluated by an experienced radiologist. The control group consisted of consecutive patients without inflammatory arthritis. RESULTS: Exactly 459 patients (204 RA, 200 SpA, and 55 PsA) and 78 patients in the control group were included in the study. CCJ involvement was detected in 101 (49.5%) RA, 53 (26.5%) SpA, 10 (18.2%) PsA, and 4 patients (5.1%) in the control group (p < 0.001). The odontoid process was one of the main targets, especially in RA patients (69 (33.8%)), which was significantly higher than in the SpA, PsA, and control groups. Although vertical subluxation (VS) was numerically higher in the RA and SpA groups compared to the control group, VS-related brainstem compression was relatively uncommon: 6 (2.9%) in RA, 1 (0.5%) in AS, and none in the PsA and control groups. CONCLUSION: CCJ involvement can often be detected in patients with inflammatory arthritis, especially in RA and SpA patients. The odontoid process is the main target of inflammation.

Objective: Autologous iliac bone is commonly used as a bone graft material to achieve solid fusion in craniocervical junction (CVJ) surgery. However, the developing iliac bone of children is less than ideal as a bone graft material. The matured rib bone of children presents a potential substitute material for iliac bone. The aim of this study was to evaluate the efficacy of autologous rib grafts for craniocervical junction surgery in children. Methods: The outcomes of 10 children with abnormalities of the craniocervical junction who underwent craniocervical junction surgery between January 2020 and December 2022 were retrospectively reviewed. All patients underwent posterior fusion and internal fixation surgery with autologous rib grafts. Pre- and post-operative images were obtained and clinical follow-ups were conducted to evaluate neurological function, pain level, donor site complications, and bone fusion rates. Results: All surgeries were successful. During the 8- to 24-month follow-up period, all patients achieved satisfactory clinical results. Computed tomography at 3–6 months confirmed successful bone fusion and regeneration of the rib defect in all patients with no neurological or donor site complications. Conclusion: Autologous rib bone is a safe and effective material for bone grafting in craniocervical junction fusion surgery for children that can reduce the risks of donor site complications and increase the amount of bone graft, thereby achieving a higher bone fusion rate. [ABSTRACT FROM AUTHOR]

Objective: To identify, quantify and compare clinical and concurrent imaging findings of occipital hypoplasia (OH), syringomyelia (SM) and atlanto-occipital overlapping (AO) in dogs with or without brachycephaly. Methods: A focused systematic search for literature was performed in the Web of Science™, PubMed and Google Scholar databases. Both authors screened and classified the identified articles using EndNote and appraised the articles using the Critical Appraisal Skills Program checklists. The main clinical and concurrent imaging features were extracted and evaluated for coexistence of OH, SM, AO, and other imaging findings. Results: Thirty-one articles were included in this minireview. For articles focusing on descriptions of OH, SM and AO, 249 dogs had at least one of these conditions, and 3 of these 249 dogs (1%) had coexistence of all three conditions. For articles focusing on descriptions of the dogs, OH, SM, and AO were identified in 552/19/11/11, 574/2/0/6, and 100/0/0/0 small brachycephalic, small non-brachycephalic, large brachycephalic, and large non-brachycephalic breeds, respectively. For all small brachycephalic dogs, the percentages of affected animals were 40% for OH (p = 0.01), 42% for SM (p < 0.01) and 7% for AO (p = 0.033). The number of dogs having AO and clinical symptoms is low (n = 5). Conclusion: OH, SM and AO are more likely to affect small dogs. AO might be limited to small brachycephalic breeds owing to the geometry of the craniocervical junction. Hence, AO alone might not lead to SM. In individual dogs, readers should carefully interpret the clinical relevance of OH or AO in the absence of SM. [ABSTRACT FROM AUTHOR]

Purpose: This study aims to develop an accessible stepwise management algorithm for pediatric presentations of occipital condyle fractures (OCFs) based on a systematic review of the published literature regarding diagnostic evaluation, treatment, and outcomes. Methods: A systematic review of the literature was conducted on PubMed to locate English language studies reporting on the management of pediatric OCFs. Data extraction of clinical presentation, management strategies, imaging, and treatment outcome was performed. Results: A total of 15 studies reporting on 38 patients aged 18 years and younger presenting with OCFs were identified. Loss of consciousness (LOC), depressed level of consciousness, neck pain, decreased neck range of motion (ROM), and cranial nerve injury were the most common presenting symptoms. Diagnostic imaging included radiographs, computed tomography (CT) scans, magnetic resonance imaging (MRI), and functional radiographs to assess cervical stability. Treatment options varied and included soft collar, hard collar, and halo vest. All studies resulted in a complete healing of the OCF, with resolution of associated pain. Conclusion: The proposed treatment algorithm suggests a framework for the management of pediatric OCFs based on the available evidence (levels of evidence: 3, 4). This review of the literature indicated that a stepwise approach should be utilized in the management of isolated pediatric OCFs. [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]

The occurrence of dural arteriovenous fistulas (DAVFs) at the craniocervical junction (CCJ) is an uncommon vascular malformation. The diagnosis and treatment of CCJ DAVFs present a formidable challenge. This study aims to investigate the effect of endovascular embolization and microsurgery on improving patient prognosis. This retrospective study included patients diagnosed with CCJ DAVFs who received treatment at the First Affiliated Hospital of Fujian Medical University between January 2000 and January 2023. The clinical records, imaging data, and treatment methods were obtained from the hospital's medical record system. The patients were classified into microsurgery and embolization groups based on the surgical technique employed for treatment. The primary outcome measures were surgical-associated neurological dysfunction (SAND) and long-term neurological outcomes. The Cox proportional hazard regression was utilized to determine hazard ratios and 95% confidence intervals (CI) to assess the relationship between treatment methods and prognosis. Kaplan-Meier survival analysis was employed to evaluate the incidence of SAND in both cohorts. This study recruited 46 patients with an average age of 53.72 ± 13.83 years. In the microsurgery group, there were 12 cases (26.1%) observed. While in the embolization group, there were 34 cases (73.9%). Of these patients, 16 (34.8%) experienced SAND after treatment. In the microsurgery group, there were 8 cases (75.0%), while in the embolization group, only 8 cases (23.5%) were reported. Specifically, the embolization group exhibited a significantly lower risk of SAND [adjusted hazard ratio = 0.259, 95% CI = 0.096–0.700; P = 0.008)] compared to the microsurgery group. Additionally, the combined Borden grade 2–3 was found to be significantly associated with SAND (adjusted hazard ratio = 3.150, 95% CI = 1.132–8.766; P = 0.028). The results of the Kaplan-Meier survival analysis indicated a statistically significant difference in the occurrence of favorable functional outcomes between the 2 groups (log-rank P = 0.0081). CCJ DAVFs are uncommon disorders characterized by a diverse range of clinical manifestations. The functional prognosis of endovascular treatment may be superior to microsurgery. [ABSTRACT FROM AUTHOR]

Purpose: Bridging veins (BVs) serve as a route of pial venous reflux, and its anatomy is essential to understand the pathophysiology of dural arteriovenous shunts (dAVSs) around the craniocervical junction (CCJ) (from the jugular foramen level to the atlantal level). However, the anatomical variations of the BVs and their proximal connections remained poorly elucidated. This study aimed to radiologically investigate the anatomy of the bridging veins around CCJ and discuss the clinical significance of these BVs in the dAVS. Methods: We investigated normal venous anatomy of the BVs from the jugular foramen level to the atlantal level using preoperative computed tomography digital subtraction venography in patients undergoing elective neurosurgery. BVs affected by the dAVSs in the same region were also evaluated. The three types of dAVS, craniocervical junction, anterior condylar, and proximal sigmoid sinus, were investigated. Results: We identified six BV groups: superolateral, anterolateral, lateral, posterior, inferolateral, and inferoposterior. The superolateral and inferolateral groups, connected with the proximal sigmoid sinus and suboccipital cavernous sinus, respectively, were the largest groups. Each group has a specific downstream venous connection. The association with dVASs was observed only in the inferolateral group, which was typically the sole venous drainage in most dAVSs at the CCJ. Conclusion: We reported detailed anatomy of BVs from the jugular level to the atlantal level, which enhanced our understanding of the pathophysiology of dAVSs in the corresponding region. [ABSTRACT FROM AUTHOR]

Background: Dural arteriovenous fistulas (DAVFs) at the craniocervical junction (CCJ) involving the first spinal nerve represent a particularly rare and challenging subtype of DAVFs, with holocord myelopathy secondary to cerebrospinal DAVFs being an exceedingly rare presentation. Case Description: We report the case of a 70-year-old woman who presented with progressive paraparesis over 2 weeks. Initial magnetic resonance imaging (MRI) of the spine showed extensive holocord myelopathy, leading to a misdiagnosis of inflammatory myelopathy and subsequent inappropriate steroid treatment at a local hospital, which exacerbated her neurological symptoms. On transfer to our institution and further evaluation with MRI and magnetic resonance angiography, a lower thoracic DAVF was initially suspected. However, comprehensive spinal angiography failed to localize the fistula, prompting cranial angiography, which ultimately identified a DAVF at the CCJ along the C1 nerve root, supplied by a small radiculomeningeal branch of the left vertebral artery. Successful management involved coagulation of the proximal draining vein, with follow-up imaging confirming complete fistula obliteration and resolution of the holocord edema. Conclusion: This case highlights the diagnostic and therapeutic challenges associated with DAVFs at the CCJ, particularly when presenting with holocord myelopathy. It underscores the importance of a high index of suspicion and the need for timely, accurate diagnosis and intervention to prevent permanent spinal cord damage in such rare and complex cases. [ABSTRACT FROM AUTHOR]

ObjectiveTo identify, quantify and compare clinical and concurrent imaging findings of occipital hypoplasia (OH), syringomyelia (SM) and atlanto-occipital overlapping (AO) in dogs with or without brachycephaly.MethodsA focused systematic search for literature was performed in the Web of Science™, PubMed and Google Scholar databases. Both authors screened and classified the identified articles using EndNote and appraised the articles using the Critical Appraisal Skills Program checklists. The main clinical and concurrent imaging features were extracted and evaluated for coexistence of OH, SM, AO, and other imaging findings.ResultsThirty-one articles were included in this minireview. For articles focusing on descriptions of OH, SM and AO, 249 dogs had at least one of these conditions, and 3 of these 249 dogs (1%) had coexistence of all three conditions. For articles focusing on descriptions of the dogs, OH, SM, and AO were identified in 552/19/11/11, 574/2/0/6, and 100/0/0/0 small brachycephalic, small non-brachycephalic, large brachycephalic, and large non-brachycephalic breeds, respectively. For all small brachycephalic dogs, the percentages of affected animals were 40% for OH (p = 0.01), 42% for SM (p

Arteriovenous fistulas (AVFs) at the craniocervical junction (CCJ) are uncommon conditions with complex angioarchitecture. The objective of this study was to identify the angioarchitectural features of CCJ-AVF that were predictive of clinical presentation and neurological function. The study encompassed a total of 68 consecutive patients with CCJ-AVF at two neurosurgical centers between 2014 and 2022. Additionally, a systematic review was conducted, including 68 cases with detailed clinical data obtained via PubMed database spanning 1990 to 2022. Clinical and imaging data were collected and pooled together to analyze factors associated with subarachnoid hemorrhage (SAH), myelopathy, and modified Rankin scale (mRS) at presentation. The mean age of the patients was 54.5 ± 13.1 years, with 76.5% of them being male. The most common feeding arteries were V3-medial branches (33.1%), and drainage was frequently through the anterior or posterior spinal vein/perimedullary vein (72.8%). SAH was the most common presentation (49.3%), and an associated aneurysm was identified as a risk factor for SAH (adjusted OR, 7.44; 95%CI, 2.89–19.15). Anterior or posterior spinal vein/perimedullary vein (adjusted OR, 2.78; 95%CI, 1.00–7.72) and male gender (adjusted OR, 3.76; 95%CI, 1.23–11.53) were associated with higher risk for myelopathy. Myelopathy at presentation was an independent risk factor for unfavorable neurological status (adjusted OR per score, 4.73; 95%CI, 1.31–17.12) in untreated CCJ-AVF. The present study identifies risk factors associated with SAH, myelopathy, and unfavorable neurological status at presentation in patients with CCJ-AVF. These findings may help treatment decisions for these complex vascular malformations. [ABSTRACT FROM AUTHOR]

The craniocervical junction (CCJ) is a complex anatomical structure comprising the occiput, the atlas, and the axis. The CCJ plays an important role in maintaining stability, providing protection, and supporting neurovascular structures. The CCJ can be affected by a wide range of congenital variants and traumatic, degenerative, inflammatory, and tumoral pathologies. This pictorial review the normal anatomy of the CCJ and presents the most common anatomical variants and pathologic conditions affecting the CCJ. [ABSTRACT FROM AUTHOR]

"Whiplash," a term describing the severe acceleration and deceleration forces applied to the head, craniocervical junction (CCJ), and cervical spine during trauma, is one of the most frequent mechanisms of injury to the CCJ. The CCJ is a complex region at the transition of the cranium and the cervical spine, essential for maintaining craniocervical stability. In whiplash injuries, the CCJ may be compromised due to underlying ligamentous or, less frequently, osseous, intravertebral disk and/or muscular lesions. Imaging is crucial in detecting acute lesions but may also play a role in the follow-up of chronic pathology because soft tissue lesions and progressive disk pathology could contribute to a whiplash-associated disorder. [ABSTRACT FROM AUTHOR]

Objective: Schwannomas of the first and second nerve roots are rare neurosurgical entities, harboring specific surgical features that make surgical resection particularly challenging and deserve specifics dissertations. This study is a retrospectively analysis of 14 patients operated in two different neurosurgical centers: the San Filippo Neri Hospital of Rome and the Federal Centre of Neurosurgery of Tjumen. Materials and Methods: In the last 6 years, 14 patients underwent neurosurgical resection of high cervical (C1–C2) schwannomas, in two different neurosurgical centers. Patients data regarding clinical presentation, radiological findings, and surgical results were retrospectively analyzed. Results: The mean age was 50 years (range 13–74), the follow-up mean duration was 30 ± 8.5 (range 24–72 months), and there was no significant differences among different tumor locations (intradural, extradural, and dumbbell). Surgical results were excellent: gross total resection was achieved in all cases and there were no intraoperative complications or postoperative mortality. All patients presented postoperative clinical improvement except one who remained stable. Karnofsky performance status, at the last follow-up, confirmed a global clinical improvement. No vertebral artery (VA) injury neither spinal instability occurred; nerve root sacrifice was reported in one case. Conclusions: Neurosurgical treatment of C1–C2 schwannomas is associated with good outcomes in terms of extent of resection and neurological function. In particular, dumbbell shape and VA involvement do not represent limitations to achieve complete tumor resection and good clinical outcome. In conclusion, microsurgery represents the treatment of choice for C1–C2 schwannomas. [ABSTRACT FROM AUTHOR]

Introduction: Upper cervical (UC) chiropractic has a more than 70-year history of pursuing empirical research, making many consider UC examination as the 'gold standard' for evaluating the UC spine. Applied Kinesiology utilises Vertebral Challenge Technique (VCT) as one tool to determine the presence and direction vertebral subluxation. Objectives: The purpose of this research is to present a preliminary assessment of the accuracy of the AK VCT when applied to the UC spine by comparing it to established UC protocols, which provide an excellent reference for comparison.. Methods: Patients selected for this study underwent a normal initial evaluation, consisting of history, history based exam, manual muscle testing exam, and UC examination. Additionally, VCT was applied to the CCJ and results for atlas laterality and rotation were recorded. Pre-adjustment VCT results were compared to UC radiographic measurements to determine agreement between these two evaluation procedures and determine the accuracy of VCT when applied to the CCJ. Results: Twenty-seven patients were included in this study. Multifactorial analysis showed that when compared to UC radiographic pre-adjustment measurements, pre-adjustment VCT showed an agreement range of 3.7%-47.6%. Post-adjustment agreement increased to 96.3% Conclusion: The lack of agreement with pre-adjustment radiographic, orthogonal model measurements of the CCJ suggests that pre-adjustment VCT applied to the atlas may not be an effective method for determining the direction of correction to reduce CCJ misalignment. However, due to the dramatic increase in agreement postadjustment, more investigation is warranted. [ABSTRACT FROM AUTHOR]

Schwannomas overall account for approximately 8% of primary brain tumors, with the majority of them arising from the vestibular nerves. 1,2 Non-vestibular schwannomas are considered rare, particularly ones arising from the accessory nerve, constituting only around 4% of craniovertebral junction schwannomas. 3,4 The far lateral approach and its variations is an important tool in the armamentarium of skull base neurosurgeons. It allows adequate exposure for accessing ventral and ventrolateral lesions of the craniocervical junction. 5-13 A 60-year-old female patient presented with a 3-month history of difficulty walking and progressive right-sided weakness. Magnetic resonance imaging demonstrated an extra-axial solid lesion at the craniocervical junction with significant enhancement on post-contrast imaging. The lesion was ventrolateral to the medulla, causing compression, displacement, and peritumoral edema. The patient consented to the procedure and underwent a far lateral suboccipital craniotomy with C1 hemilaminectomy in a lateral position. Tumor origins were identified at the left accessory nerve rootlet. The patient's postoperative course was uneventful. Follow-up magnetic resonance imaging revealed gross total resection and complete resolution of hemiparesis 3 months after the surgery. Microsurgical resection of tumors at the craniocervical junction is challenging. Preoperative planning and tailoring the approach are essential in the decision-making process to safely perform surgery. This video demonstrates, in detail, the steps, relevant anatomy, and technical nuances for accessory nerve schwannoma ressmoval. To the best of our knowledge, this is the first operative video showing the resection of a pure accessory nerve schwannoma with compression of the medulla. Under our institutional ethical review board regulations, approval was not necessary. [ABSTRACT FROM AUTHOR]

This study was conducted to investigate the clinical and anatomical importance of the relevant region from the perspective of surgical approaches by determining the morphometric analysis of the craniocervical junction and foramen magnum (FM) region and determining their distances from important anatomical points. This research was carried out with 59 skulls found at the Anatomy Laboratories of Erciyes and Ankara Medipol University. Metric measurements of FM and condyle, FM shape, condyle-fossa relationship, and pharyngeal tubercle (PT) were made in mm-based dry bone samples of unknown age and sex. The distance between the anterior notches and the FM was 87.01±4.35, the distance between the anterior notches and the PT was 77.70±4.24, the distance between the PT-sphenooccipital junction was 13.23±2.42, and the FM index was 81.86±7.47. The anteroposterior and transverse lengths of FM were determined as 33.80±2.99 and 27.72±2.30, respectively. The morphometric and morphological data available regarding the craniocervical junction showed significant differences between populations. Comprehensive knowledge of this topic will provide a better approach to treat Arnold Chiari Malformation, FM meningiomas, and other posterior cranial fossa lesions. Therefore, we believe that FM and craniocervical junction morphology will be a guide not only for anatomists, but also for radiologists, neurosurgeons, ENT surgeons, and orthopedists. [ABSTRACT FROM AUTHOR]

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Study Design. Retrospective cohort study. Objective. To verify the clinical efficacy of a novel transoral stepwise release technique (TSRT) for the treatment of irreducible atlantoaxial dislocations (IAAD). Summary of Background Data. Anterior release for IAAD remains challenging, with a 3.2 times higher complication rate than posterior release. However, there are some patients who cannot achieve successful reduction from a posterior approach and require the higher-risk anterior release. Our work presents a novel anterior release technique that aims to minimize iatrogenic injury and associated complications from an anterior release. Materials and Methods. IAAD cases who were treated with TSRT were retrospectively studied. Primary outcomes included fusion rate, complications, and neurological function over the course of a minimum 1-year follow-up. Radiographic differences between preoperative and postoperative imaging were also considered. A preoperative prediction model for the actual release grade was developed using multivariate logistic regression based on demographic factors and the craniovertebral abnormalities identified on preoperative images, evaluating the need for highergrade TSRT release. Results. We included 201 IAAD cases, with 42% (84/201) demonstrating degeneration of the atlantoaxial joint or anterior-hook-like dens. The reduction was achieved in all cases, with 80% (160/201) of cases only requiring relatively low-grade or grade I types TSRT release. Degeneration of the atlantoaxial joint was significantly associated with the need for higher-grade TSRT release (odds ratio:16.68, CI: 2.91-94.54, P = 0.002). The overall complication rate was 4.5% (9/201). Over the course of follow-up, the fusion rate reached 98.5%, and the American Spinal Injury Association and Japanese Orthopedic Association scores were significantly improved to 97.28 and 16.25 (P < 0.01 and P < 0.01), respectively. Conclusion. This study demonstrated that our novel TSRT anterior release technique demonstrated complication rates similar to those published in the literature for posterior release. TSRT can be used as an alternative to posterior release techniques for refractory cases or when a posterior approach is not considered viable. [ABSTRACT FROM AUTHOR]

Background Surgical treatment of ventral and ventrolateral lesions of the craniocervical junction are among the most challenging neurosurgical pathologies to treat. Three surgical techniques, the far lateral approach (and its variations), the anterolateral approach, and the endoscopic far medial approach can be used to approach and resect lesions in this area. Objective The aim of the study is to examine the surgical anatomy of three skull base approaches to the craniocervical junction and review surgical cases to better understand the indications and possible complications for each of these approaches. Methods Cadaveric dissections with standard microsurgical and endoscopic instruments were performed for each of the three surgical approaches, and key steps and surgically relevant anatomy were documented. Six patients with appropriate pre-, post-, and intraoperative imaging and video documentation are presented and discussed accordingly. Results Based on our institutional experience, all three approaches can be utilized to safely and effectively approach a wide variety of neoplastic and vascular pathology. Unique anatomical characteristics, lesion morphology and size, and tumor biology should all be considered when determining the optimal approach. Conclusion Preoperative assessment of surgical corridors with 3D illustrations helps to define the best surgical corridor. 360 degree knowledge of the anatomy of craniovertebral junction allows safe surgical approach and treatment of ventral and ventrolateral located lesions using one of the three approaches. [ABSTRACT FROM AUTHOR]

The craniocervical junction (CCJ) is a complex region. Rarely, arteriovenous fistulas (AVFs) can occur in the CCJ region. Currently, it is accepted that CCJ AVFs should only refer to AVFs at the C1-C2 levels. It is reasonable to assume that high cervical CCJ AVFs are being referred to when discussing CCJ AVFs. High cervical CCJ AVFs can be divided into the following four types: dural AVF, radicular AVF, epidural AVF and perimedullary AVF. Until now, it was difficult to understand high cervical CCJ AVFs and provide a proper treatment for them. Therefore, an updated review of high cervical CCJ AVFs is necessary. In this review, the following issues are discussed: the definition of high cervical CCJ AVFs, vessel anatomy of the CCJ region, angioarchitecture of high cervical CCJ AVFs, treatment options, prognoses and complications. Based on the review and our experience, we found that the four types of high cervical CCJ AVFs share similar clinical and imaging characteristics. Patients may present with intracranial hemorrhage or congestive myelopathy. Treatment, including open surgery and endovascular treatment (EVT), can be used for symptomatic AVFs. Most high cervical CCJ AVFs can be effectively treated with open surgery. EVT remains challenging due to a high rate of incomplete obliteration and complications, and it can only be performed in superselective AVFs with simple angioarchitecture. Appropriate treatment can lead to a good prognosis. [ABSTRACT FROM AUTHOR]

Dural arteriovenous fistulas at the craniocervical junction (CCJ DAVFs) are a rare vascular disease. Endovascular treatment (EVT) and microsurgery are the primary treatment modalities for CCJ DAVFs. However, incomplete treatment or complications may occur after treatment because of the anatomical complexity. We analyzed the neurosurgical treatment experiences of CCJ DAVFs to recommend suitable classification and treatment options. CCJ DAVFs were anatomically classified into three types according to the feeding arteries and their relationships with the anterior spinal (ASAs) and lateral spinal arteries (LSAs). Type 1 was fed by the radiculomeningeal artery from the vertebral artery and was not associated with the ASA or LSA. Type 2 was fed by the radiculomeningeal artery, and the radicular artery supplied the LSA near the fistula point. Type 3 had the characteristics of type 1 or type 2 CCJ DAVFs, except the ASA also contributed to the fistula. There were 5, 7, and 4 cases of type 1, type 2, and type 3 CCJ DAVFs, respectively. EVT was attempted in 12 patients, of whom only 1 (type 1) was completely cured without complications. Nine cases had residual lesions after EVT, and two had spinal cord infarction due to occlusion of the LSA. Fourteen patients underwent microsurgical treatment. In all 14 cases, CCJ DAVFs were completely obliterated after microsurgery. In cases of type 1 CCJ DAVF, both microsurgical treatment and EVT may be considered. However, for type 2 and 3 CCJ DAVFs, microsurgery may be a superior treatment modality. [ABSTRACT FROM AUTHOR]

Asymptomatic craniocervical junction arteriovenous fistulas (CCJ AVFs) are rare and, thus, a consensus has not yet been reached regarding the indication of surgical interventions. This retrospective multicenter cohort study investigated the risks associated with surgery for asymptomatic CCJ AVFs and discussed the indication of surgical interventions. Using data from 111 consecutive patients with CCJ AVFs registered with the Neurospinal Society of Japan between 2009 and 2019, we analyzed the treatment, complications, and outcomes of 18 patients with asymptomatic CCJ AVF. The median age of the patient cohort was 68 years (37–80 years), and there were 11 males and 7 females. Diagnoses were 14 patients with dural AVF, one perimedullary AVF, one radicular AVF, one epidural AVF, and one bilateral dural and epidural AVF. Initial treatment included direct surgery in 12 patients, endovascular treatment in four, and conservative treatment in two. Among 16 patients, three complications (18.7%) occurred: spinal cord infarction associated with the surgical procedure, cerebral infarction associated with intraoperative angiography, and mortal medullary hemorrhage after endovascular treatment followed by open surgery. Complete occlusion was achieved in all 12 patients in the direct surgery group and in one out of four in the endovascular treatment group. Given the risk of serious complications associated with asymptomatic CCJ AVF and the fact that no case of asymptomatic CCJ AVF became symptomatic in this study, prophylactic surgery for asymptomatic CCJ AVF should be carefully considered. [ABSTRACT FROM AUTHOR]

The craniocervical junction (CCJ) of humans and other vertebrates is a developmental restless region. Due to complex phylogenetic and ontogenetic processes, many anatomical variations can be found in that transitional area. Therefore, newly described variants must be registered, named, and classified into existing concepts explaining their genesis. This study aimed to describe and classify anatomical peculiarities that have not or rarely been reported on before in the literature. This study is based on the observation, analysis, classification, and documentation of three rare phenomena of three different human skull bases and upper cervical vertebrae, which come from the body donor program of the RWTH Aachen. As a result, three osseous phenomena (accessory ossicles, spurs, and bridges) at the CCJ of three different body donors could have been documented, measured, and interpreted. Due to extensive collecting efforts, careful maceration, and accurate observation, it is still possible to add new phenomena to the long list of Proatlas‐manifestations. Further on, it could have been shown again that these manifestations can cause damage to the elements of the CCJ due to altered biomechanic conditions. Finally, we have succeeded in showing that phenomena can exist that can imitate the presence of a Proatlas‐manifestation. Here, a precise differentiation between Proatlas‐based supernumerary structures and the results of fibroostotic processes is necessary. [ABSTRACT FROM AUTHOR]

Objective: Schwannomas of the first and second nerve roots are rare neurosurgical entities, harboring specific surgical features that make surgical resection particularly challenging and deserve specifics dissertations. This study is a retrospectively analysis of 14 patients operated in two different neurosurgical centers: the San Filippo Neri Hospital of Rome and the Federal Centre of Neurosurgery of Tjumen. Materials and Methods: In the last 6 years, 14 patients underwent neurosurgical resection of high cervical (C1–C2) schwannomas, in two different neurosurgical centers. Patients data regarding clinical presentation, radiological findings, and surgical results were retrospectively analyzed. Results: The mean age was 50 years (range 13–74), the follow-up mean duration was 30 ± 8.5 (range 24–72 months), and there was no significant differences among different tumor locations (intradural, extradural, and dumbbell). Surgical results were excellent: gross total resection was achieved in all cases and there were no intraoperative complications or postoperative mortality. All patients presented postoperative clinical improvement except one who remained stable. Karnofsky performance status, at the last follow-up, confirmed a global clinical improvement. No vertebral artery (VA) injury neither spinal instability occurred; nerve root sacrifice was reported in one case. Conclusions: Neurosurgical treatment of C1–C2 schwannomas is associated with good outcomes in terms of extent of resection and neurological function. In particular, dumbbell shape and VA involvement do not represent limitations to achieve complete tumor resection and good clinical outcome. In conclusion, microsurgery represents the treatment of choice for C1–C2 schwannomas.

Background and purpose: The purpose of this study was to identify and classify the different types of anterior atlanto-occipital membrane complex injuries on MRI and evaluate for the presence, size, and location of a prevertebral effusion on the preceding CT exams. Materials and methods: Patients who suffered an anterior atlanto-occipital membrane complex injury were identified retrospectively utilizing Nuance mPower software. An anatomic-based description of the location of the anterior atlanto-occipital membrane complex injury was recorded along with any additional osteoligamentous trauma of the craniocervical junction. The preceding cervical CT exams for these patients were reviewed for the presence and location of a prevertebral effusion. Results: Fifty patients were identified with an acute, post-traumatic anterior atlanto-occipital membrane complex injury. Three distinct patterns of anterior atlanto-occipital membrane complex injury were observed. Nineteen patients demonstrated increased STIR signal with disruption of the anterior atlanto-occipital membrane, ten patients demonstrated increased STIR signal with disruption of the anterior atlanto-axial membrane, and twenty-one patients demonstrated increased STIR signal with disruption of both the anterior atlanto-occipital membrane and anterior atlanto-axial membrane. An effusion at the C1-C2 level was present in greater than 90% of patients with anterior atlanto-occipital membrane complex injury. Conclusions: The presence of a craniocervical prevertebral effusion on CT in trauma patients may raise suspicion for an injury to the anterior atlanto-occipital membrane complex and potentially trigger additional investigation with cervical MRI. [ABSTRACT FROM AUTHOR]

Osteogenesis Imperfecta (OI) is a disease that causes bone fragility and deformities, affecting both the cranial base and the craniocervical junction, and may lead to other neurological disorders. A retrospective cross-sectional study was carried out based on cephalometric analysis of the cranial base in a sample of patients with OI, in lateral skull radiographs and magnetic resonance imaging (MRI), comparing them with a sample of age-matched controls. When the different variables of the craniocervical junction were analyzed, significance was found in comparisons with the different age groups. All measurements of the variables studied stabilized as growth progressed. For most of the variables, the severity of the disease influences the measurements of the skull base, with statistically significant differences. Both age and severity of the disease are factors that directly influence the anatomy of the craniocervical junction in these patients and may serve as indicators in the early detection and prevention of other derived alterations. [ABSTRACT FROM AUTHOR]

Background: The formation of a dural arteriovenous fistula (DAVF) at the craniocervical junction is rare. Such a fistula can be fed by the branches of the vertebral and external carotid arteries. The lateral spinal artery can branch from the vertebral artery. The feeders of a DAVF at the craniocervical junction are often diagnosed on preoperative angiography, and there is little reported evidence on the intraoperative diagnosis of the lateral spinal artery. Case description: An 84-year-old man presented with motor weakness and sensory disturbance of the lower extremities. Edematous changes in the medulla oblongata and cervical spinal cord were observed on magnetic resonance imaging. Cerebral angiography revealed a DAVF fed by a branch of the vertebral artery, with a shunting point located in the dura of the right condyle; the main drain was the anterior spinal vein. The DAVF drain was surgically obliterated to prevent hemorrhagic events and improve neurological symptoms. Intraoperatively, an artery branching from the feeder of the DAVF was identified and preserved. The patient had a good postoperative course, and the neurological symptoms were ameliorated. Follow-up cerebral angiography revealed proximal branching of the lateral spinal artery from the feeding artery of the DAVF. Conclusion: A lateral spinal artery was identified intraoperatively while a DAVF at the craniocervical junction was obliterated. This suggests that preoperative imaging should be carefully reviewed, and endovascular procedures should consider such possibilities to avoid adverse ischemic outcomes. [ABSTRACT FROM AUTHOR]

Background: Occipitocervical fusion (OCF) is a procedure performed for multiple upper cervical pathologies. A common postprocedural complication of OCF is dysphagia, which has been linked to the narrowing of the pharyngeal space due to fixation in a hyper-flexed angle. Postoperative dysphagia is linked to reduced quality of life, prolonged hospital stay, aspiration pneumonia, and increased mortality. This has led to investigations of the association between sagittal radiographic angles and dysphagia following OCF. Methods: A systematic review of the literature was performed to explore the current evidence regarding cervical sagittal radiographic measurements and dysphagia following OCF. A search strategy was carried out using the PubMed, Embase, and Web of Science databases from their dates of inception until August 2022. Only original English-language studies were considered. Moreover, studies had to include the correlation between dysphagia and at least one radiographic measurement in the sagittal plane. Results: The search and subsequent selection process yielded eight studies that were included in the final review, totaling 329 patients in whom dysphagia had been assessed and graded. The dysphagia score by Bazaz et al. (Spine 27, 22:2453–2458, 2002) was used most often. The pooled incidence of dysphagia, in the early postoperative period, was estimated at 26.4%. At long-term follow-up (range: 17–72 months), about one-third of patients experienced resolution of symptoms, which resulted in a long-term post-OCF dysphagia incidence of 16.5%. Across the studies included, six different radiographic parameters were used to derive several measures which were repeatedly and significantly associated with the occurrence of dysphagia. Conclusions: The high incidence of postoperative dysphagia following OCF warrants close monitoring of patients, especially in the short-term postoperative period. These patients may be assessed through standardized tools where the one by Bazaz et al. was the most commonly used. Moreover, there are several radiographic measurements that can be used to predict the occurrence of dysphagia. These findings may serve as a basis for strategies to prevent the occurrence of dysphagia after OCF. [ABSTRACT FROM AUTHOR]

The far lateral approach provides wide surgical access to the lower third of the clivus, pontomedullary junction, and anterolateral foramen magnum and rarely requires craniovertebral fusion. The most common indications for this approach are posterior inferior cerebellar artery and vertebral arteryaneurysms, brainstem cavernous malformations, and tumors anterior to the lower pons and medulla, including meningiomas of the anterior foramen magnum, schwannomas of the lower cranial nerves, and intramedullary tumors at the craniocervical junction. We provide a stepwise description of how we perform the far lateral approach, as well as how to combine the far lateral approach with other skull base approaches, including the subtemporal transtentorial approach, for lesions involving the upper clivus; the posterior transpetrosal approach, for lesions involving the cerebellopontine angle and/or petroclival region; and/or lateral cervical approaches, for lesions involving the jugular foramen or carotid sheath regions. [ABSTRACT FROM AUTHOR]

Background: Craniocervical junction (CCJ) vascular abnormalities can be challenging to treat because of the surrounding density of critical neurovascular anatomy. Although most dural arteriovenous fistulas (dAVFs) are now treated with endovascular surgery, dAVFs near the CCJ are often better suited for microsurgical obliteration with precise vascular control. Methods: We describe our microsurgical approach to treating dAVFs at the CCJ. This includes a far-lateral approach with a small incision centered over the transverse process of the atlas and circumferential skeletonization of the vertebral artery in addition to clipping the fistula to limit lesion recurrence. Conclusions: Definitive microsurgical treatment of CCJ dAVFs can be accomplished using a minimally invasive approach. [ABSTRACT FROM AUTHOR]

• DAVF of the craniocervical junction can have different venous drainage patterns. • The venous drainage can be inferior, superior or both. • The anatomy of the DAVF predicts the clinical presentation of the patient. Patients with superior drainage are more likely to present with a hemorrhage whereas patients with inferior drainage tend to present with myelopathy. • In our paper, we propose a novel classification system in which DAVF of the craniocervical junction can be grouped into three types and for each type, we present a treatment paradigm. • Microsurgery remains an effective treatment option. There is an emerging role of transvenous embolization particularly for superior draining DAVF. Dural arteriovenous fistulas (DAVF) of the craniocervical junction (CCF) are an uncommon entity with the following venous drainage pattern: inferior, superior and mixed. Patients may present with subarachnoid hemorrhage, myelopathy or brainstem dysfunction. CCJ DAVF can be treated with microsurgery or with transarterial and transvenous embolization, depending on the venous drainage pattern. We present our institutional experience of treating CCJ DAVFs along with a systematic review of the literature. Six patients with CCJ DAVF were treated at our institution over five years. Data was collected using electronic medical record review. Systematic review was performed on CCJ DAVF using the PubMed database from 1990 to 2021. We characterized venous drainage patterns, treatment choices, and outcomes to create a classification system. 50 case reports, consisting of 115 patients, were included in our review. 61 (53.0 %) patients had inferior drainage while 32 (27.8 %) patients had superior drainage and 22 (19.2 %) patients had mixed venous drainage. Patients with inferior drainage had the fistulous connection at the foramen magnum while patients with superior drainage had a fistulous connection at C1-C2 (p value = 0.026). Patients with inferior drainage were more likely to present with myelopathy while patients with superior drainage presented with hemorrhage (p value = 0.000). Classifying the venous drainage pattern is essential in making treatment decision. Transvenous embolization works best with large superior venous drainage. If endovascular treatment is not an option, then surgical clipping can achieve successful cure. Transarterial embolization is a reasonable option in cases with a large arterial feeder. [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.

Craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula are rare cerebrovascular lesions. While their pathophysiology is different, both conditions can cause intracranial hemorrhage attributable to venous congestion. We present, to our knowledge, the first case report of craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula presenting concomitantly in separate locations with subarachnoid hemorrhage. This case appears to have been due to increased venous hypertension caused by a merging of the venous drainage of the 2 lesions, resulting in hemorrhage. [ABSTRACT FROM AUTHOR]