craniocervical junction
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2022 ◽  
Vol 3 (3) ◽  

BACKGROUND Posterior atlantoaxial dislocations (i.e., complete anterior odontoid dislocation) without C1 arch fractures are a rare hyperextension injury most often found in high-velocity trauma patients. Treatment options include either closed or open reduction and optional spinal fusion to address atlantoaxial instability due to ligamentous injury. OBSERVATIONS A 60-year-old male was struck while on his bicycle by a truck and sustained an odontoid dislocation without C1 arch fracture. Imaging findings additionally delineated a high suspicion for craniocervical instability. The patient had neurological issues due to both a head injury and ischemia secondary to an injured vertebral artery. He was stabilized and transferred to our facility for definitive neurosurgical care. LESSONS The patient underwent a successful transoral digital closed reduction and posterior occipital spinal fusion via a fiducial-based transcondylar, C1 lateral mass, C2 pedicle, and C3 lateral mass construct. This unique reduction technique has not been recorded in the literature before and avoided potential complications of overdistraction and the need for odontoidectomy. Furthermore, the use of bone fiducials for navigated screw fixation at the craniocervical junction is a novel technique and recommended particularly for placement of technically demanding transcondylar screws and C2 pedicle screws where pars anatomy is potentially unfavorable.


Neurospine ◽  
2021 ◽  
Vol 18 (4) ◽  
pp. 741-748
Author(s):  
Daimon Shiraishi ◽  
Yusuke Nishimura ◽  
Isaac Aguirre-Carreno ◽  
Masahito Hara ◽  
Satoshi Yoshikawa ◽  
...  

Objective: The purpose of this study is to find the clinical and radiographic characteristics of traumatic craniocervical junction (CCJ) injuries requiring occipitocervical fusion (OC fusion) for early diagnosis and surgical intervention.Methods: We retrospectively reviewed 12 patients with CCJ injuries presenting to St. Michaels Hospital in Toronto who underwent OC fusion and looked into the following variables; (1) initial trauma data on emergency room arrival, (2) associated injuries, (3) imaging characteristics of computed tomography (CT) scan and magnetic resonance imaging (MRI), (4) surgical procedures, surgical complications, and neurological outcome.Results: All patients were treated as acute spinal injuries and underwent OC fusion on an emergency basis. Patients consisted of 10 males and 2 females with an average age of 47 years (range, 18–82 years). All patients sustained high-energy injuries. Three patients out of 6 patients with normal BAI (basion-axial interval) and BDI (basion-dens interval) values showed visible CCJ injuries on CT scans. However, the remaining 3 patients had no clear evidence of occipitoatlantal instability on CT scans. MRI clearly described several findings indicating occipitoatlantal instability. The 8 patients with normal values of ADI (atlantodens interval interval) demonstrated atlantoaxial instability on CT scan, however, all MRI more clearly and reliably demonstrated C1/2 facet injury and/or cruciate ligament injury.Conclusion: We advocate measures to help recognize CCJ injury at an early stage in the present study. Occipitoatlantal instability needs to be carefully investigated on MRI in addition to CT scan with special attention to facet joint and ligament integrity.


2021 ◽  
Vol 12 ◽  
Author(s):  
Heidi Arponen ◽  
Marjut Evälahti ◽  
Outi Mäkitie

BackgroundBiallelic mutations in the non-coding RNA gene RMRP cause Cartilage-hair hypoplasia (CHH), a rare skeletal dysplasia in which the main phenotypic characteristic is severe progressive growth retardation.ObjectiveThis study compared the cranial dimensions of individuals with CHH to healthy subjects.MethodsLateral skull radiographs of 17 patients with CHH (age range 10 to 59 years) and 34 healthy individuals (age range 10 to 54 years) were analyzed for relative position of the jaws to skull base, craniofacial height and depth, as well as vertical growth pattern of the lower jaw, anterior cranial base angle, and the relationship between the cervical spine and skull base.ResultsWe found that the length of the upper and lower jaws, and clivus were significantly decreased in patients with CHH as compared to the controls. Anterior cranial base angle was large in patients with CHH. Basilar invagination was not found.ConclusionThis study found no severe craniofacial involvement of patients with CHH, except for the short jaws. Unexpectedly, mandibular deficiency did not lead to skeletal class II malocclusion.Clinical ImpactAlthough the jaws were shorter in patients with CHH, they were proportional to each other. A short posterior cranial base was not associated with craniocervical junction pathology.


2021 ◽  
Vol 2 (23) ◽  
Author(s):  
Haggai Suisa ◽  
Jean Francois Soustiel ◽  
Yuval Grober

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.


2021 ◽  
pp. 1-8

OBJECTIVE Craniocervical junction (CCJ) arteriovenous fistulas (AVFs) are treated using neurosurgical or endovascular options; however, there is still no consensus on the safest and most effective treatment. The present study compared the treatment results of neurosurgical and endovascular procedures for CCJ AVFs, specifically regarding retreatment, complications, and outcomes. METHODS This was a multicenter cohort study authorized by the Neurospinal Society of Japan. Data on consecutive patients with CCJ AVFs who underwent neurosurgical or endovascular treatment between 2009 and 2019 at 29 centers were analyzed. The primary endpoint was the retreatment rate by procedure. Secondary endpoints were the overall complication rate, the ischemic complication rate, the mortality rate, posttreatment changes in the neurological status, independent risk factors for retreatment, and poor outcomes. RESULTS Ninety-seven patients underwent neurosurgical (78 patients) or endovascular (19 patients) treatment. Retreatment rates were 2.6% (2/78 patients) in the neurosurgery group and 63% (12/19 patients) in the endovascular group (p < 0.001). Overall complication rates were 22% and 42% in the neurosurgery and endovascular groups, respectively (p = 0.084). Ischemic complication rates were 7.7% and 26% in the neurosurgery and endovascular groups, respectively (p = 0.037). Ischemic complications included 8 spinal infarctions, 2 brainstem infarctions, and 1 cerebellar infarction, which resulted in permanent neurological deficits. Mortality rates were 2.6% and 0% in the neurosurgery and endovascular groups, respectively (p > 0.99). Two patients died of systemic complications. The percentages of patients with improved modified Rankin Scale (mRS) scores were 60% and 37% in the neurosurgery and endovascular groups, respectively, with a median follow-up of 23 months (p = 0.043). Multivariate analysis identified endovascular treatment as an independent risk factor associated with retreatment (OR 54, 95% CI 9.9–300; p < 0.001). Independent risk factors associated with poor outcomes (a postoperative mRS score of 3 or greater) were a pretreatment mRS score of 3 or greater (OR 13, 95% CI 2.7–62; p = 0.001) and complications (OR 5.8; 95% CI 1.3–26; p = 0.020). CONCLUSIONS Neurosurgical treatment was more effective and safer than endovascular treatment for patients with CCJ AVFs because of lower retreatment and ischemic complication rates and better outcomes.


2021 ◽  
Vol 2 (21) ◽  
Author(s):  
Matthew A. Liu ◽  
Julian L. Gendreau ◽  
Joshua J. Loya ◽  
Nolan J. Brown ◽  
Amber Keith ◽  
...  

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


2021 ◽  
Author(s):  
yuan chenghua ◽  
jian guan ◽  
yueqi du ◽  
zeyu fang ◽  
xinyu wang ◽  
...  

Abstract Background No prior reports have focused on spinal cord injury (SCI) characteristics or inflammation after destruction of the blood-spinal cord barrier by syringomyelia. To compare the difference of syringomyelia-related central SCI between craniocervical junction (CCJ) and post-traumatic syringomyelia (PTS) before and after decompression. Methods Between 2015 and 2019, 106 CCJ, 26 CCJ revision and 15 PTS patients (mean history of symptoms 71.5 ± 94.3, 88.9 ± 85.5 and 32.3 ± 48.9 months). The symptom courses were analysed with the ASIA, Klekamp and Samii scoring systems and Kaplan-Meier statistics for neurological changes. The mean follow-up was 20.7 ± 6.2, 21.7 ± 8.8 and 34.8 ± 19.4 months. Results Compared with the other group, the interval time after PTS was longer, but the natural history of syringomyelia was shorter (P=0.0004, 0.0173, respectively). The initial symptoms were usually paraesthesia (P=0.258), and the symptoms were mainly hypoesthesia (P=0.006), abnormal muscle strength (P=0.004), gait (P<0.0001) and abnormal urination (P<0.0001). SCI associated with PTS was more severe than that CCJ related (P=0.003). The cavities in the PTS group were primarily located at the thoracolumbar level, which was different from those in the cervical-thoracic segment at the CCJ. The rate of syrinx/cord was more than 75% (P=0.009), and the intradural adhesions tended to be more severe (P<0.0001). However, there were no significant differences in peripheral blood inflammation markers (PBIM) or long-term clinical efficacy except for the RBC (P=0.042). Conclusion The natural history of PTS tends to progress faster and is more severe than CCJ related. PBIM had no distinguishing effect on the difference in inflammation of syringomyelia except for the RBC. The predictive value of NLR for syringomyelia-related inflammation except in the acute phase was negative.


2021 ◽  
pp. 101419
Author(s):  
Abhijith Bathini ◽  
Joshua Lucas ◽  
Mohanad Sulaiman ◽  
Anwesha Dubey ◽  
Ahmad Mohammad Kassem ◽  
...  

2021 ◽  
Vol 12 ◽  
pp. 501
Author(s):  
Valérie Nicole Elise Schuermans ◽  
Jasper van Aalst ◽  
Alida A. Postma ◽  
Anouk Y. J. M. Smeets

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


Author(s):  
Alessandra D'Amico ◽  
Teresa Perillo ◽  
Renato Cuocolo ◽  
Lorenzo Ugga ◽  
Fabiola Di Dato ◽  
...  

Alagille syndrome (ALGS) is a multisystemic disease caused by mutations in genes of Notch pathway, which regulates embryonic cell differentiation and angiogenesis. Clinically, ALGS is characterized by cholestasis, cardiac defects, characteristic facial features, skeletal and ophthalmologic abnormalities. The aim of this review is to illustrate neuroradiological findings in ALGS, which are less well-known and prevalent, including cerebrovascular anomalies (such as aneurysms, dolichoectasia, Moyamoya syndrome and venous peculiarities), Chiari 1 malformation, craniosynostosis, intracranial hypertension, and vertebral anomalies (namely butterfly vertebra, hemivertebra, and craniocervical junction anomalies). Rarer cerebral midline malformations and temporal bone anomalies have also been described.


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