Motion-related vascular abnormalities at the craniocervical junction: illustrative case series and literature review.

The craniocervical junction (CCJ) functions within a complicated regional anatomy necessary to protect and support vital neurovascular structures. In select instances, vascular pathology can be attributed to this complicated interplay of motion and structure found within this narrow space. The authors report 3 cases of complex vascular pathology related to motion at the CCJ and detail the management of these cases. Two cases involved posterior circulation vascular compression syndromes, and one case involved a vascular anomaly and its relation to aneurysm formation and rupture. The patient in Case 1 was a 66-year-old man with a history of syncopal episodes resulting from the bilateral vertebral artery becoming occluded when he rotated his head. Successful microsurgical decompression at the skull base resulted in patent bilateral vertebral artery V3 segments upon head movement in all directions. The patient in Case 2 was a 53-year-old woman who underwent elective resection of a right temporal meningioma and who experienced postoperative drowsiness, dysphagia, and mild right-arm ataxia. Subsequent MRI demonstrated bilateral posterior inferior cerebel-lar artery (PICA) strokes. Cerebral angiography showed a single PICA, of extradural origin, supplying both cerebellar hemispheres. The PICA exhibited dynamic extradural compression when the patient rotated her head; the bilateral PICA strokes were due to head rotation during surgical positioning. In Case 3, a 37-year-old woman found unconscious in her home had diffuse subarachnoid hemorrhage and evidence of a right PICA aneurysm. A right far-lateral craniectomy was performed for aneurysm clipping, and she was found to have a dissecting aneurysm with an associated PICA originating extradurally. There was a shearing phenomenon of the extradural PICA along the dura of the foramen magnum, and this microtraumatic stress imposed on the vessel resulted in a dissecting aneurysm. This series of complex and unusual cases highlights the authors' understanding of vascular pathology of the CCJ and its management.

Unilateral fixation for treatment of occipitocervical instability in children with congenital vertebral anomalies of the craniocervical junction.

OBJECT Patients with occipitocervical (OC) instability from congenital vertebral anomalies (CVAs) of the craniocervical junction (CCJ) often have bony abnormalities that make instrumentation placement difficult. Within this patient population, some bilateral instrumentation constructs either fail or are not feasible, and a unilateral construct must be used. The authors describe the surgical management and outcomes of this disorder in patients in whom unilateral fixation constructs were used to treat OC instability. METHODS From a database of OC fusion procedures, the authors identified patients who underwent unilateral fixation for the management of OC instability. Patient characteristics, surgical details, and radiographic outcomes were reviewed. In each patient, CT scans were performed at least 4 months after surgery to evaluate for fusion. RESULTS Eight patients with CVAs of the CCJ underwent unilateral fixation for the treatment of OC instability. For 4 patients, the procedure occurred after a bilateral OC construct failed or infection forced hardware removal. For the remainder, it was the primary procedure. Two patients required reoperation for hardware revision and 1 developed nonunion requiring revision of the bone graft. Ultimately, 7 patients demonstrated osseous fusion on CT scans and 1 had a stable fibrous union. CONCLUSIONS These findings demonstrate that a unilateral OC fixation is effective for the treatment of OC instability in children with CVAs of the CCJ in whom bilateral screw placement fails or is not feasible.

Computed tomography evaluation of the normal craniocervical junction craniometry in 100 asymptomatic patients.

OBJECT: Most of the craniometric relationships of the normal craniocervical junction (CCJ), especially those related to angular craniometry, are still poorly studied and based on measurements taken from simple plain radiographs. In this study, the authors performed a craniometric evaluation of the CCJ in a population without known CCJ anomalies. The purpose of the study was to evaluate the normal CCJ craniometry based on measurements obtained from CT scans. METHOD: The authors analyzed 100 consecutive CCJ CT scans obtained in adult patients who were admitted at their tertiary hospital for treatment of non-CCJ conditions between 2010 and 2012. A total of 17 craniometrical measurements were performed, including the relation of the odontoid with the cranial base, the atlantodental interval (ADI), the clivus length, the clivus-canal angle (CCA)-the angle formed by the clivus and the upper cervical spine, and the basal angle. RESULTS: The mean age of the 100 patients was 50.6 years, and the group included 52 men (52%) and 48 women (48%). In 5 patients (5%), the tip of the odontoid process was more than 2 mm above the Chamberlain line, and in one of these 5 patients (1% of the study group). it was more than 5 mm above it. One patient had a Grabb-Oakes measurement above 9 mm (suggesting ventral cervicomedullary encroachment). The mean ADI value was 1.1 mm. The thickness of the external occipital protuberance ranged from 7.42 to 22.36 mm. The mean clivus length was 44.74 mm, the mean CCA was 153.68° (range 132.32°-173.95°), and the mean basal angle was 113.73° (ranging from 97.06°-133.26°). CONCLUSIONS: The data obtained in this study can be useful for evaluating anomalies of the CCJ in comparison with normal parameters, potentially improving the diagnostic criteria of these anomalies. When evaluating CCJ malformations, one should take into account the normal ranges based on CT scans, with more precise bone landmarks, instead of those obtained from simple plain radiographs.

Transarterial embolization of radicular arteriovenous fistula at the craniocervical junction.

Craniocervical junction arteriovenous fistula (CCJ AVF) is a rare vascular disorder. Direct surgery for CCJ AVF is generally reported to have better outcome compared to endovascular treatment. However, no certain consensus has been obtained so far. We report a case of radicular CCJ AVF treated by transarterial embolization that resulted in a good outcome. A 69-year-old man presented with subarachnoid hemorrhage primarily in the posterior cranial fossa. Based on digital subtraction angiography showed radicular CCJ AVF with varix. Transarterial embolization was performed with n-butyl-2-cyanoacrylate on day 17 after onset and successfully cured. The neurovascular anatomy of CCJ AVF is complicated, but endovascular treatment may be a treatment option with detailed understanding of angioarchitecture and selective endovascular procedure.

Estimation of the rupture point of the craniovertebral junction intradural arteriovenous fistula with vessel wall magnetic resonance image and its pathological findings: A case report.

Background: Arteriovenous fistulas (AVFs) of the craniocervical junction (CCJ) and intradural AVFs are often associated with aneurysms and varics, and it is sometimes difficult to identify the ruptured point on radiological images. We report a case in which vessel wall magnetic resonance image (VW-MRI) was useful for identifying the ruptured point at the CCJ AVF. Case Description: A 70-year-old man presented with a sudden onset of headache. He had Glasgow Coma Scale E4V5M6, world federation of neurosurgical societies (WFNS) Grade I. Fisher group 3 subarachnoid hemorrhage and hydrocephalus were found on head computed tomography. Cerebral angiography showed a spinal AVF at the C1 level of the cervical spine. Magnetic resonance image-enhanced motion sensitized driven equilibrium (MSDE-method showed an enhancing effect in part of the AVF draining vein, but the vascular architecture of this lesion was indeterminate. We performed continuous ventricular drainage for acute hydrocephalus and antihypertensive treatment. Cerebral angiography was performed 30days after the onset of the disease, and was revealed an aneurysmal structure in a portion of the AVF draining vein, which VW-MRI initially enhanced. On the 38th day after onset, he underwent direct surgery to occlude the AV fistula and dissect the aneurysmal structure. Histopathology showed that the aneurysmal structure was varices with lymphocytic infiltration, and hemosiderin deposition was observed near the varices. Conclusion: Recently, VW-MRI has been reported to show an association between the enhancement of varices in dural AVF and rupture cases. VW-MRI, especially the enhanced MSDE method, may be useful in estimating the ruptured point in arteriovenous shunt disease.

Atlanto-occipital dislocation in a child: a challenging diagnosis. Illustrative case.

BACKGROUND: Atlanto-occipital dislocation (AOD) is a highly unstable injury of the osseoligamentous complex at the craniocervical junction that is more common in children. Its diagnosis remains a challenging process that must integrate clinical presentation and radiological criteria. OBSERVATIONS: A 9-year-old child presented with severe craniocervical trauma (Glasgow Coma Scale score 6) and cardiorespiratory arrest on-site. Prompt resuscitation on-site and transfer to the university hospital were performed, and a computed tomography (CT) scan showed a subarachnoid hemorrhage around the brainstem and a retroclival hematoma. Most of the radiological criteria on CT scans for AOD were negative, except for the occipital condyle-C1 interval, and further imaging with magnetic resonance imaging permitted the diagnosis of AOD with rupture of both the tectorial membrane and the transverse ligament. Occipital-cervical Oc-C1-2 fixation was performed. The neurological outcome was excellent, with full recovery 6 months after the trauma. LESSONS: AOD should be suspected in all high-intensity trauma in children, especially if the clinical presentation includes cardiorespiratory arrest and other brainstem and/or upper cervical cord symptoms along with premedullary subarachnoid hemorrhage. Understanding the ligamentous nature of the injury resulting in "normal" radiographs or CT scans is important to avoid underdiagnosing AOD, which can have detrimental consequences.

Efficacy of intraarterial indocyanine green videoangiography in surgery for arteriovenous fistula at the craniocervical junction in a hybrid operating room: illustrative cases.

BACKGROUND: Sufficient understanding of the angioarchitecture of an arteriovenous fistula (AVF) at the craniocervical junction (CCJ) is crucial to surgical treatment but is often difficult because of the complex vascular anatomy. Intraarterial indocyanine green (ICG) videoangiography has emerged as a more useful option for understanding the vascular anatomy than intravenous ICG videoangiography. This report describes two cases of CCJ AVFs successfully treated by surgery using intraarterial ICG videoangiography and describes the efficacy of this technique. OBSERVATIONS: Case 1 involved a 71-year-old man presenting with tetraparesis after sudden onset of severe headache due to subarachnoid hemorrhage (SAH). Digital subtraction angiography (DSA) demonstrated CCJ epidural AVF. Intraarterial ICG videoangiography revealed the drainer, which had been difficult to identify. The AVF disappeared after disconnection of the drainer. Case 2 involved a 68-year-old man presenting with severe headache due to SAH. DSA showed multiple AVFs at the CCJ and cerebellar tentorium. Intraarterial ICG videoangiography demonstrated concomitant perimedullary AVF and dural AVF at the CCJ. All AVFs disappeared postoperatively. LESSONS: Intraarterial ICG videoangiography was useful for definitive diagnosis of CCJ AVF, facilitating identification of feeders and drainers with bright and high phase contrast and allowing repeated testing to confirm flow direction.

Evaluation of using the Anderson-Montesano and the Tuli classifications in pediatric patients with occipital condyle fractures.

BACKGROUND: Occipital condyle fractures (OCFs) in patients before 18 years of age are rare. Classifications of OCF are based on the CT images of the cranio-cervical junction (CCJ) and MRI. The Anderson-Montesano and Tuli classifications are the types which are most commonly used in these cases. Classification of OCFs allows the implementation of OCF treatment. The aim of this study was to evaluate the effectiveness of using the OCF classification in pediatric patients based on the analysis of our own cases. METHODS: During the years 2013-2020, 6 pediatric patients with OCFs, aged 14-18, have been treated. Two patients with unstable fracture III according to Anderson-Montesano and IIB according to Tuli were treated with the halo-vest. Additionally, one patient presenting neurological symptoms and with an associated C1 fracture was qualified for the halo-vest stabilization as well. The other patients were treated with a Minerva collar. We evaluated the results 6 months after completing the OCF treatment using the Neck Disability Index (NDI) and SF-36 questionnaires. Confidence intervals for the mean values were verified using the MeanCI function (from the R library DescTools) for both classical and bootstrap methods. RESULTS: Based on NDI results, we have obtained in our patients an average of 4.33/45 points (2-11) and 9.62% (4.4-24.4). Based on the SF-36 questionnaire, we obtained an average of 88.62% (47.41-99.44). CONCLUSION: The Anderson-Montesano and Tuli's classifications of OCF can be used to assess the stability of OCF in adolescents, but both classifications should be used simultaneously. CT and MR imaging should be used in diagnosing OCFs, whereas CT allows assessing therapeutic outcomes in OCF.

Asymptomatic spinal dural arteriovenous fistula: case series and systematic review.

OBJECTIVE: Spinal dural arteriovenous fistulas (SDAVFs) commonly present with symptoms of myelopathy due to venous congestion in the spinal cord; asymptomatic SDAVFs are rarely encountered. To elucidate the clinical characteristics of asymptomatic SDAVFs, the authors present 5 new cases of asymptomatic SDAVF and report the results of their systematical review of the associated literature. METHODS: Five databases were systematically searched for all relevant English-language articles on SDAVFs published from 1990 to 2018. The clinical features and imaging findings of asymptomatic SDAVFs were collected and compared with those of symptomatic SDAVFs. RESULTS: Twenty cases, including the 5 cases from the authors' experience, were found. Asymptomatic SDAVFs were more prevalent in the cervical region (35.0%); cervical lesions account for only 2% of all symptomatic SDAVFs. The affected perimedullary veins tended to drain more cranially (50.0%) than caudally (10.0%). Four cases of asymptomatic SDAVF became symptomatic, 1 case spontaneously disappeared, and the remaining 15 cases were unchanged or surgically treated. CONCLUSIONS: The higher prevalence of asymptomatic SDAVFs in the cervical spine might be a distinct feature of asymptomatic SDAVFs. Given that venous congestion is the pathophysiology of a symptomatic SDAVF, abundant collateral venous pathways and unique flow dynamics of the CSF in the cervical spine might prevent asymptomatic cervical SDAVFs from becoming symptomatic. In cases in which venous congestion is avoidable, not all asymptomatic SDAVFs will become symptomatic.

Clipping of an anterior spinal artery aneurysm using an endoscopic fluorescence imaging system for craniocervical junction epidural arteriovenous fistula: technical note.

The authors report the case of a 78-year-old man with a craniocervical junction epidural arteriovenous fistula who presented with subarachnoid hemorrhage from a ruptured anterior spinal artery (ASA) aneurysm. Because endovascular embolization was difficult, a posterolateral approach was chosen and a novel endoscopic fluorescence imaging system was utilized to clip the aneurysm. The fluorescence imaging system provided clear and magnified views of the ventral spinal cord simultaneously with the endoscope-integrated indocyanine green videoangiography, which helped safely obliterate the ASA aneurysm. With the aid of this novel imaging system, surgeons can appreciate and manipulate complex vascular pathologies of the ventral spinal cord through a posterolateral approach, even when the lesion is closely related to the ASA.

The occipitoatlantal capsular ligaments are the primary stabilizers of the occipitoatlantal joint in the craniocervical junction: a finite element analysis.

OBJECTIVEThere is contradictory evidence regarding the relative contribution of the key stabilizing ligaments of the occipitoatlantal (OA) joint. Cadaveric studies are limited by the nature and the number of injury scenarios that can be tested to identify OA stabilizing ligaments. Finite element (FE) analysis can overcome these limitations and provide valuable data in this area. The authors completed an FE analysis of 5 subject-specific craniocervical junction (CCJ) models to investigate the biomechanics of the OA joint and identify the ligamentous structures essential for stability.METHODSIsolated and combined injury scenarios were simulated under physiological loads for 5 validated CCJ FE models to assess the relative role of key ligamentous structures on OA joint stability. Each model was tested in flexion-extension, axial rotation, and lateral bending in various injury scenarios. Isolated ligamentous injury scenarios consisted of either decreasing the stiffness of the OA capsular ligaments (OACLs) or completely removing the transverse ligament (TL), tectorial membrane (TM), or alar ligaments (ALs). Combination scenarios were also evaluated.RESULTSAn isolated OACL injury resulted in the largest percentage increase in all ranges of motion (ROMs) at the OA joint compared with the other isolated injuries. Flexion, extension, lateral bending, and axial rotation significantly increased by 12.4% ± 7.4%, 11.1% ± 10.3%, 83.6% ± 14.4%, and 81.9% ± 9.4%, respectively (p ≤ 0.05 for all). Among combination injuries, OACL+TM+TL injury resulted in the most consistent significant increases in ROM for both the OA joint and the CCJ during all loading scenarios. OACL+AL injury caused the most significant percentage increase for OA joint axial rotation.CONCLUSIONSThese results demonstrate that the OACLs are the key stabilizing ligamentous structures of the OA joint. Injury of these primary stabilizing ligaments is necessary to cause OA instability. Isolated injuries of TL, TM, or AL are unlikely to result in appreciable instability at the OA joint.

Assessment of craniocervical motion in Down syndrome: a pilot study of two measurement techniques.

OBJECTIVE: Hypermobility of the craniocervical junction (CCJ) in patients with Down syndrome (DS) is common. Whereas atlantoaxial (C1-2) hypermobility is well characterized, occipitoatlantal (Oc-C1) laxity is recognized but poorly defined. A clear understanding of the risks associated with DS-related hypermobility is lacking. Research efforts to address the topic of axial cervical spine instability in the patient with DS require a reliable and reproducible means of assessing CCJ mobility. The authors conducted a pilot study comparing two methods of quantifying motion of the CCJ on dynamic (flexion/extension) plain radiographs: the delta-condyle-axial interval (ΔCAI) and the delta-basion-axial interval (ΔBAI) methods. METHODS: Dynamic radiographs from a cohort of 10 patients with DS were evaluated according to prescribed standards. Independent movement of Oc-C1, C1-2, and Oc-C2 was calculated. Interrater and intrarater reliability for CCJ mobility was then calculated for both techniques. RESULTS: Measurement using the ΔCAI technique had excellent fidelity with intraclass correlation coefficients (ICCs) of 0.77, 0.71, and 0.80 for Oc-C1, C1-2, and Oc-C2, respectively. The ΔBAI technique had lower fidelity, yielding respective ICCs of 0.61, 0.65, and 0.50. CONCLUSIONS: This pilot study suggests that ΔCAI is a superior measurement technique compared to ΔBAI and may provide reliable assessment of the mobility of the CCJ on dynamic radiographs in the pediatric patient with DS. The use of reliable and reproducible measurement techniques strengthens the validity of research derived from pooled database efforts.

Results of 1940 embolizations for dural arteriovenous fistulas: Japanese Registry of Neuroendovascular Therapy (JR-NET3).

OBJECTIVE: Embolization is the most common treatment for dural arteriovenous fistulas (dAVFs). A retrospective, multicenter observational study was conducted in Japan to clarify the nature, frequency, and risk factors for complications of dAVF embolization. METHODS: Patient data were derived from the Japanese Registry of Neuroendovascular Therapy 3 (JR-NET3). A total of 40,169 procedures were registered in JR-NET3, including 2121 procedures (5.28%) in which dAVFs were treated with embolization. After data extraction, the authors analyzed complication details and risk factors in 1940 procedures performed in 1458 patients with cranial dAVFs treated with successful or attempted embolization. RESULTS: Transarterial embolization (TAE) alone was performed in 858 cases (44%), and transvenous embolization (TVE) alone was performed in 910 cases (47%). Both TAE and TVE were performed in one session in 172 cases (9%). Complications occurred in 149 cases (7.7%). Thirty-day morbidity and mortality occurred in 55 cases (2.8%) and 16 cases (0.8%), respectively. Non-sinus-type locations, radical embolization as the strategy, procedure done at a hospital that performed dAVF embolization in fewer than 10 cases during the study period, and emergency procedures were independent risk factors for overall complications. CONCLUSIONS: Complication rates of dAVF embolization in Japan were acceptable. For better results, the risk factors identified in this study should be considered in treatment decisions.

Stand-alone craniocervical decompression is feasible in children with mucopolysaccharidosis type I, IVA, and VI.

BACKGROUND CONTEXT: In patients with mucopolysaccharidosis (MPS), glycosaminoglycan deposits in the dura mater and supporting ligaments cause spinal cord compression and consecutive myelopathy, predominantly at the craniocervical junction. Disease characteristics of craniocervical stenosis (CCS) in patients with MPS differ profoundly from other hereditary and degenerative forms. Because of high periprocedural morbidity and mortality, patients with MPS pose a substantial challenge to the inexperienced medical care provider. As literature remains scarce, we present our experience with a large cohort of patients with MPS treated for CCS without atlanto-occipital instrumentation. PURPOSE: The present study aimed to describe a safe and least traumatic approach for treating CCS in children with MPS, avoiding primary instrumentation. STUDY DESIGN: This is a prospective follow-up (cohort) study. PATIENT SAMPLES: We report 15 consecutive patients with CCS related to MPS, who were treated with stand-alone cervical decompression. OUTCOME MEASURES: Myelopathy was assessed using magnetic resonance imaging (MRI), somatosensory evoked potentials, and clinical evaluation. Cervical instability was evaluated using plain x-ray and MRI. The disability status is quantified using either the Karnofsky or Lansky Performance Score. METHODS: We describe 15 consecutive patients treated with craniocervical decompression. Data were collected prospectively. The mean follow-up is 6 years (5 standard deviation). The technique and treatment principles are described. RESULTS: The overall clinical outcome in this patient cohort is good (mean Karnofsky Performance Score of 80). No patient developed signs of C0-C1-C2 instability or progressive myelopathy. Restenosis occurred in seven patients, requiring a total of eight reoperations. CONCLUSIONS: Surgery in patients with MPS is associated with high morbidity and mortality of up to 4.2%. Because of the unique nature of the disease, recurring stenosis is inevitable. To shorten the procedure time and simplify the anticipated reoperation, we provide data that craniocervical decompression is feasible without the necessity of primary osteosynthesis. In the absence of craniocervical instability, decompression surgery without occipitocervical stabilization yields good postoperative results and challenges the long-standing paradigm of prophylactic craniocervical fixation.

Extreme lateral transodontoid approach to the ventral craniocervical junction: cadaveric dissection and case illustrations.

OBJECTIVE: Surgical treatment of pathological processes involving the ventral craniocervical junction (CCJ) traditionally involves anterior and posterolateral skull base approaches. In cases of bilateral extension, when lesions extend beyond the midline to the contralateral side, a unilateral corridor may result in suboptimal resection. In these cases, the lateral extent of the tumor will prevent extirpation of the lesion via anterior surgical approaches. The authors describe a unilateral operative corridor developed along an extreme lateral trajectory to the anterior aspect of the clival and upper cervical dura, allowing exposure and resection of tumor on the contralateral side. This approach is used when the disease involves the bone structures inherent to stability at the anterior CCJ. METHODS: To achieve exposure of the ventral CCJ, an extreme lateral transcondylar transodontoid (ELTO) approach was performed with transposition of the ipsilateral vertebral artery, followed by drilling of the C1 anterior arch. Resection of the odontoid process allowed access to the contralateral component of lesions across the midline to the region of the extracranial contralateral vertebral artery, maximizing resection. RESULTS: Exposure and details of the surgical procedure were derived from anatomical cadavers. At the completion of cadaveric dissection, morphometric measurements of the relevant anatomical landmarks were obtained. Illustrative case examples for approaching ventral CCJ chordomas via the ELTO approach are presented. CONCLUSIONS: The ELTO approach provides a safe and direct surgical corridor to treat complex lesions at the ventral CCJ with bilateral extension through a single operative corridor. This approach can be combined with other lateral approaches or posterior infratemporal approaches to remove more extensive lesions involving the rostral clivus, jugular foramen, and temporal bone.

Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction.

OBJECTIVE Previous reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction. METHODS The authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center. Clinical (age, sex, and associated disorders/syndromes) and radiographic (CM type, tonsillar descent, pBC2 line, clival-axial angle [CXA], syrinx length and level, and initial Cobb angle) information was evaluated to identify associations with the primary outcome: delayed thoracolumbar fusion for progressive scoliosis. RESULTS Twenty-eight patients were identified, but 4 were lost to follow-up and 1 underwent fusion within a year. Among the remaining 23 patients, 11 required fusion surgery at an average of 88.3 ± 15.4 months after SODD, including 7 (30%) who needed fusion more than 5 years after SODD. On univariate analysis, a lower CXA (131.5° ± 4.8° vs 146.5° ± 4.6°, p = 0.034), pBC2 > 9 mm (64% vs 25%, p = 0.06), and higher initial Cobb angle (35.1° ± 3.6° vs 22.8° ± 4.0°, p = 0.035) were associated with the need for thoracolumbar fusion. Multivariable modeling revealed that lower CXA was independently associated with a need for delayed thoracolumbar fusion (OR 1.12, p = 0.0128). CONCLUSIONS This investigation demonstrates the long-term outcome and natural history of CRS after SODD. The durability of the effect of SODD on CRS and curve behavior is poor, with late curve progression occurring in 30% of patients. Factors associated with CRS progression include an initial pBC2 > 9 mm, lower CXA, and higher Cobb angle. Lower CXA was an independent predictor of delayed thoracolumbar fusion. Further study is necessary on a larger cohort of patients to fully elucidate this relationship.

Angioarchitecture of arteriovenous fistulas at the craniocervical junction: a multicenter cohort study of 54 patients.

OBJECTIVE The aim of this retrospective multicenter cohort study was to assess the details of the angioarchitecture of arteriovenous fistulas (AVFs) at the craniocervical junction (CCJ) and to determine the associations between the angiographic characteristics and the clinical presentations and outcomes. METHODS The authors analyzed angiographic and clinical data for patients with CCJ AVFs from 20 participating centers that are members of the Japanese Society for Neuroendovascular Therapy (JSNET). Angiographic findings (feeding artery, location of AV shunt, draining vein) and patient data (age, sex, presentation, treatment modality, outcome) were tabulated and stratified based on the angiographic types of the lesions, as diagnosed by a member of the CCJ AVF study group, which consisted of a panel of 6 neurointerventionalists and 1 spine neurosurgeon. RESULTS The study included 54 patients (median age 65 years, interquartile range 61-75 years) with a total of 59 lesions. Five angiographic types were found among the 59 lesions: Type 1, dural AVF (22 [37%] of 59); Type 2, radicular AVF (17 [29%] of 59); Type 3, epidural AVF (EDAVF) with pial feeders (8 [14%] of 59); Type 4, EDAVF (6 [10%] of 59); and Type 5, perimedullary AVF (6 [10%] of 59). In almost all lesions (98%), AV shunts were fed by radiculomeningeal arteries from the vertebral artery that drained into intradural or epidural veins through AV shunts on the dura mater, on the spinal nerves, in the epidural space, or on the spinal cord. In more than half of the lesions (63%), the AV shunts were also fed by a spinal pial artery from the anterior spinal artery (ASA) and/or the lateral spinal artery. The data also showed that the angiographic characteristics associated with hemorrhagic presentations-the most common presentation of the lesions (73%)-were the inclusion of the ASA as a feeder, the presence of aneurysmal dilatation on the feeder, and CCJ AVF Type 2 (radicular AVF). Treatment outcomes differed among the angiographic types of the lesions. CONCLUSIONS Craniocervical junction AVFs commonly present with hemorrhage and are frequently fed by both radiculomeningeal and spinal pial arteries. The AV shunt develops along the C-1 or C-2 nerve roots and can be located on the spinal cord, on the spinal nerves, and/or on the inner or outer surface of the dura mater.

Outcomes and effectiveness of posterior occipitocervical fusion for suboccipital spinal metastases.

OBJECTIVE The incidence of suboccipital spinal metastases is rare but has increased given cancer patients' longer life expectancies. Operative treatment in this region is often challenging because of limited fixation points due to tumor lysis, as well as adjacent neural and vascular anatomy. Few studies have reported on this population of cancer patients. The purpose of this study was to evaluate clinical outcomes and complications of patients with suboccipital spinal metastases who had undergone posterior occipitocervical fixation. METHODS A single-institution database was reviewed to identify patients with suboccipital metastases who had undergone posterior-only instrumented fusion between 1999 and 2014. Clinical presentation, perioperative complications, and postoperative results were analyzed. Pain was assessed using the visual analog scale. Survival analysis was performed using a Kaplan-Meier curve. The revised Tokuhashi and the Tomita scoring systems were used for prognosis prediction. RESULTS Fifteen patients were identified, 10 men and 5 women with mean age of 64.8 ± 11.8 years (range 48-80 years). Severe neck pain without neurological deficit was the most common presentation. Primary tumors included lung, breast, bladder, myeloma, melanoma, and renal cell cancers. All tumors occurred in the axis vertebra. Preoperative Tokuhashi and Tomita scores ranged from 5 to 13 and 3 to 7, respectively. All patients had undergone occipitocervical fusion of a mean of 4.6 levels (range 2-7 levels). Median survival was 10.3 months. In all cases, neck pain markedly improved and patients were able to resume activities of daily living. The average postoperative pain score was significantly improved as compared with the average preoperative score (1.90 ± 2.56 and 5.50 ± 2.99, respectively, p = 0.01). Three patients experienced postoperative medical complications including urinary tract infection, deep vein thrombosis, myocardial infarction, and cardiac arrhythmia. In the follow-up period, no wound infections or reoperations occurred and no patients experienced spinal cord deficits from tumor recurrence. CONCLUSIONS Posterior-only occipitocervical stabilization was highly effective at relieving patients' neck pain. No instrumentation failures were noted, and no neurological complications or tumor progression causing spinal cord deficits was noted in the follow-up period.

Development and initial evaluation of a finite element model of the pediatric craniocervical junction.

OBJECT There is a significant deficiency in understanding the biomechanics of the pediatric craniocervical junction (CCJ) (occiput-C2), primarily because of a lack of human pediatric cadaveric tissue and the relatively small number of treated patients. To overcome this deficiency, a finite element model (FEM) of the pediatric CCJ was created using pediatric geometry and parameterized adult material properties. The model was evaluated under the physiological range of motion (ROM) for flexion-extension, axial rotation, and lateral bending and under tensile loading. METHODS This research utilizes the FEM method, which is a numerical solution technique for discretizing and analyzing systems. The FEM method has been widely used in the field of biomechanics. A CT scan of a 13-month-old female patient was used to create the 3D geometry and surfaces of the FEM model, and an open-source FEM software suite was used to apply the material properties and boundary and loading conditions and analyze the model. The published adult ligament properties were reduced to 50%, 25%, and 10% of the original stiffness in various iterations of the model, and the resulting ROMs for flexion-extension, axial rotation, and lateral bending were compared. The flexion-extension ROMs and tensile stiffness that were predicted by the model were evaluated using previously published experimental measurements from pediatric cadaveric tissues. RESULTS The model predicted a ROM within 1 standard deviation of the published pediatric ROM data for flexion-extension at 10% of adult ligament stiffness. The model's response in terms of axial tension also coincided well with published experimental tension characterization data. The model behaved relatively stiffer in extension than in flexion. The axial rotation and lateral bending results showed symmetric ROM, but there are currently no published pediatric experimental data available for comparison. The model predicts a relatively stiffer ROM in both axial rotation and lateral bending in comparison with flexion-extension. As expected, the flexion-extension, axial rotation, and lateral bending ROMs increased with the decrease in ligament stiffness. CONCLUSIONS An FEM of the pediatric CCJ was created that accurately predicts flexion-extension ROM and axial force displacement of occiput-C2 when the ligament material properties are reduced to 10% of the published adult ligament properties. This model gives a reasonable prediction of pediatric cervical spine ligament stiffness, the relationship between flexion-extension ROM, and ligament stiffness at the CCJ. The creation of this model using open-source software means that other researchers will be able to use the model as a starting point for research.

Skull base chordomas: clinical outcome in a consecutive series of 45 patients with long-term follow-up and evaluation of clinical and biological prognostic factors.

OBJECTIVE Skull base chordomas (SBCs) are rare dysembryogenetic invasive tumors with a variable tendency for recurrence. According to previous studies, the recurrence rate seems to be affected by both clinical variables and tumor biological features. The authors present the results of treatment of SBCs in a large series of patients and investigate the role of 1p36 chromosomal region loss of heterozygosity (LOH) as a prognostic factor. METHODS Between 1990 and 2011, 45 patients were treated for SBCs. The mean follow-up was 76 months (range 1-240 months). An LOH analysis was performed in 27 cases. Survival analysis was performed to determine clinical and biological parameters correlating with clinical outcome. RESULTS The 5- and 10-year overall survival rates were 67% and 57%, respectively. Five- and 10-year progression-free survival rates were 58% and 44%, respectively. Multivariate analysis showed that extent of resection, adjuvant radiation therapy, and absence of rhinopharynx invasion were positive independent predictors of overall survival. The latter 2 variables and a younger patient age were positive independent predictors of progression-free survival. Twenty-one patients showed 1p36 LOH. All events of recurrence and death clustered in the group of patients with 1p36 LOH; however, this biological marker was not statistically significant on multivariate analysis. CONCLUSIONS Resection is the treatment of choice in primary and recurrent SBC. Patient age, rhinopharynx invasion at diagnosis, extent of tumor removal, and postoperative radiation therapy influence SBC prognosis. Genetic analysis, even while showing interesting results, did not reveal 1p36 LOH as an independent predictor of clinical outcome.