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We investigated the mechanism underlying Chiari malformation type I (CM-I) and classified it according to the morphometric analyses of posterior cranial fossa (PCF) and craniocervical junction (CCJ). Three independent subtypes of CM-I were confirmed (CM-I types A, B, and C) for 484 cases and 150 normal volunteers by multiple analyses. CM-I type A had normal volume of PCF (VPCF) and occipital bone size. Type B had normal VPCF and small volume of the area surrounding the foramen magnum (VAFM) and occipital bone size. Type C had small VPCF, VAFM, and occipital bone size. Morphometric analyses during craniocervical traction test demonstrated instability of CCJ. Foramen magnum decompression (FMD) was performed in 302 cases. Expansive suboccipital cranioplasty (ESCP) was performed in 102 cases. Craniocervical posterolateral fixation (CCF) was performed for CCJ instability in 70 cases. Both ESCP and FMD showed a high improvement rate of neurological symptoms and signs (84.4%) and a high recovery rate of the Japanese Orthopaedic Association (JOA) score (58.5%). CCF also showed a high recovery rate of the JOA score (69.7%), with successful joint stabilization (84.3%). CM-I type A was associated with other mechanisms that caused ptosis of the brainstem and cerebellum (CCJ instability and traction and pressure dissociation between the intracranial cavity and spinal canal cavity), whereas CM-I types B and C demonstrated underdevelopment of the occipital bone. For CM-I types B and C, PCF decompression should be performed, whereas for small VPCF, ESCP should be performed. CCF for CCJ instability (including CM-I type A) was safe and effective.

A 9‐month‐old male entire Doberman Pinscher presented with acute onset of severe cervical hyperesthesia after a fall. Neurological examination revealed a normal gait with low head carriage and severe cervical hyperesthesia. A CT scan of the cervical vertebral column revealed the presence of a comminuted fracture at the dorsomedial aspect of the right occipital condyle and sclerosis of the underlying bone. Medical management was initiated consisting of an external bandage, strict rest, and pain medication. Due to the lack of clinical improvement, the dog was euthanized 2 months after diagnosis. Histopathology of the lesion was compatible with a healing fracture. [ABSTRACT FROM AUTHOR]

To our best knowledge, most of the craniometric studies on the normal craniocervical junction (CCJ), are still poorly studied and based on measurements taken from plain radiographs. In this study, the authors conducted a craniometric evaluation of the CCJ in a population without known CCJ abnormalities. The purpose of the study was to assess the normal CCJ craniometry based on measures obtained from CT scans. The authors examined 137 consecutive CCJ CT scans obtained in patients evaluated at their hospital for treatment of non-CCJ conditions between 2018 and 2019. Twelve craniometrical dimensions were conducted, including the relation of the odontoid with the cranial base, the atlantodental interval (ADI), the clivus length, and the clivus-canal angle (CCA). [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the MRI findings of the craniocervical junction ligaments and compare the 2D TSE sequence and 3D SPACE sequence on cervical spine MRI. Methods: Eighty cervical spine MRIs were retrospectively analyzed, including 2D TSE and 3D SPACE sequences. The visibility and morphology of the transverse ligament (TL) and alar ligament (AL) were evaluated by two readers using a five-point grading scale for visibility (1 = not visible, 2 = barely visible, 3 = adequately visible, 4 = good visibility, 5 = excellent visibility) and a four-point grading scale for morphology (0 = homogeneous low SI with normal thickness, 1 = high SI with normal thickness, 2 = reduced thickness, 3 = full-thickness rupture or indistinguishable from surrounding structures). The grades of the two sequences were compared. Reader agreements were measured using the percentage of exact agreement. Result: The visibility grade of TL was significantly higher in 3D, indicating better visualization. The morphology grade of TL was significantly lower in the 3D, indicating more normal-appearing ligament. The exact agreements of visibility and morphology grade of TL tended to be higher in 3D. On the other hand, there was no significant difference in visibility grade of AL between the two sequences. The morphological grade of AL was significantly higher in 3D. The agreement of the morphological grade of AL tended to be lower in 3D. Conclusion: The utilization of 3D sequences significantly improves the visibility of TL and could potentially reduce false-positive diagnoses of ligament injury. [ABSTRACT FROM AUTHOR]

Background: An arteriovenous fistula (AVF) at the craniocervical junction (CCJ) is a rare vascular malformation. Definitive diagnosis and curative treatment of CCJ AVF are challenging. Case Description: A 77-year-old man presented with subarachnoid hemorrhage. Cerebral angiography showed an AVF at the CCJ, which drained into a radicular vein. The lesion was fed by a vertebral artery, anterior and lateral spinal arteries (LSAs), and the occipital artery (OA). There were two unique structures: the LSA originating from the posterior inferior cerebellar artery of the extracranial V3 segment and the OA feeding the shunt. Curative treatment involved two steps: endovascular embolization of feeders using Onyx and surgical shunt disconnection. Feeding arteries were blackened by Onyx, which helped identify the location of the shunt. The shunt was located behind the first cervical (C1) spinal nerve, and the draining vein was confirmed on the deep side of the nerve. A clip was applied to the draining vein distal to the shunt. Tiny vessels supplying the shunt were then coagulated referring to blackened arteries. Conclusion: A radicular AVF at the CCJ along the C1 spinal nerve had unique vascular structures. Definitive diagnosis and curative treatment were achieved by combining endovascular embolization using Onyx and direct surgery. [ABSTRACT FROM AUTHOR]

Objective Spinal reconstruction after resection of invasive craniocervical junction malignancies is fraught with technical and management considerations as well as a paucity of data in the existing literature. In this study, we describe our experience with craniocervical junction malignancies, especially the influence of radiation on the need for revision spinal instrumentation. Methods We performed a retrospective chart review of all patients who underwent occipitocervical fixation between 2011 and 2019 at The University of Texas MD Anderson Cancer Center. Results Twenty-five patients had primary malignancies and 12 (30%) had metastatic tumors. Thirteen (33%) underwent a staged resection in multiple operations during their hospital stay. Tumor resection was performed in 19 patients (48%), while only stabilization was performed in 21 patients (52%). Nine patients (23%) underwent expanded endoscopic transclival approaches for tumor resection, 10 patients (25%) an extreme lateral approach, and 2 patients (5%) an anterior open approach. Eleven patients underwent early postoperative radiation therapy (within 3 months) and 8 underwent delayed radiation therapy (between 3 months and 1 year in 7 patients). The revision rate was 8%, with a median time to revision surgery of 42 months. The administration and timing of adjuvant radiation therapy relative to surgery had no significant effect on the need for instrumentation revision on log-rank and Cox regression analyses (p < 0.05). Conclusion Revision surgery was needed infrequently in our patients. Postoperative radiation therapy was not associated with hardware failure, indicating that the timing of radiation therapy should be dictated by the diagnosis and can be initiated postoperatively without delay.

Abstract A neonatal Dutch Warmblood colt was evaluated for inability to stand, incoordination and intention tremor. Despite partial improvement in clinical signs during the first 4 days of hospitalization, neurological deficits remained. Magnetic resonance imaging identified a unilateral infratentorial arachnoid cyst‐like lesion with ipsilateral compression and displacement of the cerebellar hemisphere, absent corpus collosum, polymicrogyria, suspect leukoencephalopathy, and noncompressive occipitoatlantal malformation. Improvement in clinical signs during the first 6 months of life suggests that horses can survive with complex congenital neurological malformations, but prognosis for athletic performance is poor. The accessibility of magnetic resonance imaging should improve the diagnostic accuracy of central nervous system disorders in neonatal foals in which congenital malformations are suspected. Euthanasia often is elected in foals with suspected congenital central nervous system disorders because of poor prognosis for athletic performance, limiting understanding of clinical progression in these cases.

Spinal arteriovenous fistulae (AVFs) of the craniocervical junction are rare lesions that have variable angioarchitecture. These lesions are generally characterized by arteriovenous shunting at a single fistulous point, the disconnection of which is generally curative. 1 We present a case of bilateral dural AVF at the C2 level treated with surgical ligation (Video 1). Our patient was an elderly woman who presented after cervical magnetic resonance imaging was performed as part of work-up for right-sided shoulder tingling. This study demonstrated prominent flow voids along the dorsal and ventral surfaces of the craniocervical junction, concerning for an underlying vascular malformation. Formal angiography demonstrated arteriovenous shunting into the perimedullary venous plexus from a dural branch of the vertebral artery at the C2 level. Significant retrograde cortical venous drainage was noted. She was treated with a C1 and partial C2 laminectomy and clip ligation of the right-sided fistula. Postoperative angiography confirmed obliteration of the fistula but now demonstrated persistent arteriovenous shunting from second fistulous point at the contralateral C2 level. She was then taken back to surgery for ligation of the second fistula. Instances of multiple, distinct, and synchronous arteriovenous fistulae have been rarely reported in the literature. 2 In addition to demonstrating the nuances of surgical treatment of AVFs at this location, this case demonstrates the potential for synchronous AVFs and emphasizes the importance of thorough postoperative angiography. The patient provided consent to the production of this surgical video. [ABSTRACT FROM AUTHOR]

Objective: The purpose of our study was to identify adult trauma patients with an acute C1 burst fracture, evaluate for concomitant transverse atlantal ligament (TAL) injury, and apply the modified Gehweiler and AO spine classification systems to determine the utility of these classification systems in accurately defining C1 trauma. Materials and Methods: Adult trauma patients with an acute C1 fracture were identified retrospectively using Nuance mPower software. The C1 fracture was described based on whether the fracture involved the anterior arch, posterior arch, lateral mass, medial tubercle, and/or transverse process. If follow-up cervical magnetic resonance imaging (MRI) was performed, the presence and location of an associated TAL injury was recorded. The anatomic location of the C1 burst fracture and TAL injury, if present, were compared with the descriptive classification systems outlined by Gehweiler/Dickman (modified) and the AO Spine society. Any additional osseous trauma of the skull base and C1-C2 was also recorded along with relevant clinical history and management. Results: Thirty-nine patients were identified with an acute C1 burst fracture on cervical computed tomography (CT) with seventy-seven percent of patients undergoing follow-up cervical MRI. Observed fracture patterns were divided into five distinct types based on CT findings and further subdivided based on the integrity of the transverse altantal ligament on MRI. TAL tears were observed exclusively in type 3 fractures (anterior and posterior arch fractures) and type 4 fractures (anterior arch, posterior arch, and lateral mass fractures). The modified Gehweiler classification system failed to accurately describe the anatomic location of the C1 fracture in forty-four percent of patients, whereas the AO spine was too broad and failed to accurately describe fracture location in our cohort. Conclusions: The Gehweiler and AO spine classifications demonstrated significant shortcomings in the accurate description of patients with C1 trauma. Whereas the Gehweiler system did not accurately describe the anatomic location of the various C1 fractures, the AO spine system was too broad and failed to radiologically classify fracture location. Moreover, there was a high number of patients with AO spine type B injuries without atlantoaxial translation that nevertheless required C1-C2 fusion for atlantoaxial instability. We suggest the need for an updated classification system that takes into account both the CT (fracture location) and MRI (TAL integrity) appearance of C1 trauma. An updated classification strategy will offer a radiologic standardization of C1 trauma that will aid in future research studies and help optimize patient management.

Context: Atlanto-occipital dissociation is a highly lethal ligamentous injury at the craniocervical junction (CCJ). Previous studies have described rare cases of milder forms of atlanto-occipital injury (AOI) which might be managed nonoperatively, but there is a paucity of literature on this subject. Aims: We retrospectively reviewed our institutional experience to characterize the injury patterns, treatments, and clinical courses of patients with unilateral AOI. Methods: We included patients with radiographic evidence of unilateral occipitocervical joint capsular disruption, distraction, or edema ± injury of the apical ligament, tectorial membrane, anterior atlanto-occipital membrane, posterior atlanto-occipital membrane, alar ligaments, or cruciate ligament. The long-term outcomes were gathered from medical records, and six patients were available for Neck Disability Index via phone call at the time of the study. Results: Eight patients were included in the study. The mean age was 45.1 years ± 26.5. Causes of trauma included motor vehicle collision for five patients (5/8, 62.5%), falls for two (2/8, 25), and assault for one (1/8, 12.5%). All patients had a widened condyle-C1 interval >2 mm. Three patients underwent occipitocervical fusion, one patient underwent atlantoaxial fusion, and another received subaxial fusions for other injuries. Three patients underwent no surgical intervention. All patients were seen at least once as an outpatient following hospital discharge. There were no delayed neurologic injuries or deaths. Conclusions: We propose that ligamentous injury at the CCJ functions more as a spectrum rather than dichotomous diagnosis, of which a subset can likely be safely managed nonoperatively.

Os odontoideum (OO) is a rare craniocervical anomaly that is characterized by a round ossicle separated from the C2 vertebral body. With a controversial etiology and unknown prevalence in the population, OO may be asymptomatic or present in patients with myelopathic and neurological symptoms. In this literature review, we aimed to investigate epidemiology, embryology, pathophysiology, clinical presentation, and the role of diagnostic radiography in OO. By providing an overview of different management strategies, surgical complications, and postoperative considerations for OO, our findings may guide neurosurgeons in providing proper diagnosis and treatment for OO patients. A literature review was conducted using the PubMed, EMBASE, and Scopus databases. A search using the query “os odontoideum” yielded 4319 results, of which 112 articles were chosen and analyzed for insights on factors such as etiology, clinical presentation, and management of OO. The presentation of OO varies widely from asymptomatic cases to severe neurological deficits. Asymptomatic patients can be managed either conservatively or surgically, while symptomatic patients should undergo operative stabilization. Although multiple studies show different techniques for surgical management involving posterior fusion, the diversity of such cases illustrates how treatment must be tailored to the individual patient to prevent complications. Conflicting studies and the paucity of current literature on OO highlight poor comprehension of the condition. Further understanding of the natural history of OO is critical to form evidence-based guidelines for the management of OO patients. More large-center studies are thus needed to promote accurate management of OO patients with optimal outcomes.

Tumors at the craniocervical junction represent a formidable challenge due to its intimate association with the medulla and cervical spinal cord, lower cranial nerves, and vertebral artery (VA). Foramen magnum meningioma (FMM) reparents 2% of all intracranial meningiomas and 4% of posterior fossa meningiomas. The far lateral approach is preferred for FMM with VA involvement. Here, we describe a case of a 58-year-old woman who presented with headache, nausea and vomiting and gait disturbance and was found to have large FMM encasing the dominant VA. The patient was treated with a left far lateral approach with limited condylectomy resulting in gross total resection and preservation of lower cranial nerves function.

ObjectiveThe objective of the study is to establish a new parameter that can be clearly measured on x-ray images to complement the description of the sagittal alignment of the craniocervical junction. The authors anticipate that this new parameter will enhance surgeons' understanding of the sagittal alignment of the craniocervical junction and play a positive role in the guidance of intraoperative reduction and in the evaluation of postoperative outcomes of patients with atlantoaxial instability.MethodsFrom November 2018 to June 2020, a total of 159 asymptomatic subjects who underwent frontal and lateral cervical x-ray examination in the Second Affiliated Hospital of Soochow University were included in the study. Age, gender, previous spinal trauma, and disease history of each subject were recorded. After screening, 127 effective samples were finally obtained. When taking lateral cervical radiographs, all subjects placed their neck in a neutral position and looked straight ahead with both eyes. On the obtained lateral x-ray images, a straight line was drawn from the radix to the anterior clinoid process; another line was made along the posterior edge of the C2 vertebral body; and the angle between the two lines was measured, which was defined as the “horizontal view-axial angle.” The angle formed by the tangent of the posterior edge of the C2 vertebra and C7 vertebral body is the “C2–C7 angle,” which was used to describe the curvature of the lower cervical vertebra. The normal range of horizontal view-axial angle and its relationship with C2–7 angle were evaluated.ResultsThe average C2–C7 angle of male subjects was (14.0° ± 7.4°), while that of female subjects was (11.09° ± 7.36°). The average horizontal view-axial angle of male subjects was (92.79° ± 4.52°), and that of female subjects was (94.29° ± 4.50°). Pearson correlation test showed that there was a significant negative correlation between horizontal view-axis angle and C2–C7 angle.ConclusionsFor patients with atlantoaxial instability diseases, the horizontal view-axis angle is expected to be a sagittal parameter to guide the intraoperative reduction and evaluate postoperative outcomes.

ObjectiveArteriovenous fistulas (AVFs) in the craniocervical junction (CCJ) region are a rare occurrence with special clinical manifestations. This study retrospectively reviewed patients with CCJ AVFs treated at our neurosurgical center, aiming to enhance the understanding of CCJ AVFs.MethodsA total of 113 patients with CCJ AVFs treated at our neurosurgical center between January 2013 and December 2020 were enrolled. They were grouped as patients with CCJ AVFs with spinal arterial feeders (n = 20) and patients with CCJ AVF without spinal arterial feeders (n = 93). Clinical presentation, angiographic characteristics, intraoperative findings, and treatment outcomes were analyzed.ResultsThe patients’ median age was 55 years (IQR 47.5–62 years). The proportion of males in the group without spinal arterial feeders was significantly higher (p = 0.001). Subarachnoid hemorrhage (SAH) was the most common clinical presentation, especially in the group with spinal arterial feeders (p

In Chiari Malformation Type I (CM1), low-lying tonsils obstruct the cisterna magna at the foramen magnum, thereby compromising the essential juncture between the cranial and spinal compartments. The anatomical obstruction of the cisterna magna inhibits bi-directional CSF flow as well as CSF pulse pressure equilibration between the intracranial compartment and the intraspinal compartment in response to instances of increased intracranial pressure. Less understood, however, are the roles of the spinal cord suspension structures at the craniocervical junction which lend viscoelastic support to the spinal cord and tonsils, as well as maintain the anatomical integrity of the cisterna magna and the dura. These include extradural ligaments including the myodural bridges (MDBs), as well as intradural dentate ligaments and the arachnoid framework. We propose that when these elements are disrupted by the cisterna magna obstruction, tonsillar pathology, and altered CSF dynamics, there may arise a secondary pathophysiology of compromised and dysfunctional cranio-spinal suspension in CM1. We present intraoperative images and videos captured during surgical exposure of the craniocervical junction in CM1 to illustrate this proposal. [ABSTRACT FROM AUTHOR]

Background: Symptomatic Chiari 1 malformation (CM1) is a common condition in Neurosurgery. Surgery involves hindbrain decompression and restoration of CSF flow through different surgical approaches. No Class 1 evidence exists to suggest the superiority of any of the surgical techniques. Aims: To investigate current surgical practice for symptomatic CM1 patients in the United Kingdom (UK) and determine the willingness to participate in a randomised controlled trial (RCT) comparing different surgical techniques. Methods: An electronic survey was sent to consultant members of the Society of British Neurological Surgeons and the British Chiari-Syringomyelia Group. The questions covered pre-operative and intra-operative management, presence of equipoise/uncertainty in optimal technique and willingness to participate in an RCT. Results: 98 responses were received. 67% operate on adults. 30% on adult and paediatric patients. There is variation in routine pre-operative use of: ICP monitoring (18%), flexion/extension x-rays (16%), venography (20%) and ophthalmology assessment (26%). 18% of neurosurgeons would not offer foramen magnum decompression when the presenting symptom is only refractory cough/sneeze headache. 15% routinely perform bony decompression alone in adults vs 8% in children. In 68% of adult cases, durotomy is performed routinely (46% of them leave the dura open, 54% perform a type of duroplasty) and 16% routinely resect the cerebellar tonsils. Only 17% leave the dura open in children. The most common indicators for durotomy are syringomyelia and intra-operative ultrasound findings. 61% believe there is equipoise/uncertainty in the optimal strategy for decompression and would be willing to participate in an RCT. Comments also mention the heterogeneity of CM1 and that treatment should be tailored to each patient. Conclusion: There is wide variation in pre- and intra-operative management of CM1 patients in the UK and the majority of neurosurgeons would be willing to participate in an RCT comparing bony decompression alone vs dural opening with/without duroplasty. [ABSTRACT FROM AUTHOR]

Purpose: Although the craniocervical junction has a complex anatomical structure associated with clinical diseases, its ventral venous network has not been well studied. This study aimed to clarify the extracranial ventral venous structure at the craniocervical junction. Methods: Head computed tomography digital subtraction venography (CT-DSV) images of 273 patients (age 6 months to 93 years) taken at our department were retrospectively analyzed. We analyzed the frequency and anatomical features of the venous channels, as well as their upstream and downstream connections with the surrounding channels at the ventral craniocervical junction, from the level of the hypoglossal canal to the second cervical vertebra. Results: In 54% of the cases, the vein descended from the anterior condylar confluence, running medially along the basioccipital and fusing with its counterpart in the midline at the level of the atlanto-occipital membrane. Furthermore, 24% of this vein was connected caudally to the anterior external vertebral venous plexus. We also identified venous channels, either as a sole vein or venous plexus, on the tip of the odontoid process (10%), which has not been well described previously. The vein around the odontoid process was connected to several veins, including the aforementioned vein anterior to the condyle and the anterior internal vertebral venous plexus. Conclusions: CT-DSV analysis revealed a detailed venous architecture ventral to the craniocervical junction. Venous structures identified in this study may be involved in diseases in this area. [ABSTRACT FROM AUTHOR]

In den ersten Lebenswochen kommt der kraniozervikalen Region bei der Entstehung und Behandlung von infantilen Haltungsasymmetrien und Plagiozephalien eine besondere Bedeutung zu. In der vorliegenden Sekundäranalyse werden die Ergebnisse befundorientierter osteopathischer Behandlungen auf die passive hochzervikale Rotation und den Grad der Haltungsasymmetrie gegenüber physiotherapeutischen Behandlungen nach dem Vojta-Konzept vorgestellt. Im Rahmen einer klinischen Interventionsstudie wurden durch 10 Osteopath* innen 45 Säuglinge mit Haltungsasymmetrie und lagebedingter Plagiozephalie in zwei Experimentalgruppen aufgeteilt. Die Interventionsgruppe A (OMT-Gruppe) erhielt über einen Zeitraum von 3–5 Wochen 1× wöchentlich eine befundorientierte osteopathische und die Interventionsgruppe B (Vojta-Gruppe) 1–2× in der Woche eine physiotherapeutische Behandlung nach dem Vojta-Konzept. 3 Monate nach der ersten Behandlung erfolgte eine Verlaufskontrolle. Ermittelt wurden u. a. Veränderungen des zervikalen Flexions-Rotations-Tests und des Asymmetriescores. Die erhobenen Befunde wurden im Inner- und im Zwischengruppenvergleich statistisch analysiert. Beide Behandlungsmethoden verbessern den Grad der Asymmetrie (OMT-Gruppe: p = 3,7E-21; Vojta-Gruppe: p = 1,4E-05) und die passive hochzervikale Rotation signifikant. In der Gesamtrotation (FRT sum) erreicht die OMT-Gruppe dabei bereits zur 2. Behandlung (T2: p = 0,013) und die Vojta-Gruppe zum Follow-up-Termin (T4: p = 0,035) eine Signifikanz. Eine isolierte Analyse der Behandlungen durch zwei erfahrene Kinderosteopathen deuten auf einen signifikanten Unterschied in der Verbesserung des Flexions-Rotations-Tests (T3: p = 2,8E-03) gegenüber der Vojta-Gruppe hin. Sowohl physiotherapeutische Behandlungen nach dem Vojta-Konzept, als auch befundorientierte osteopathische Behandlungen können im Rahmen der Analyse als effektive Behandlungsmöglichkeiten angesehen werden. Die osteopathische Intervention erzielt dabei einen effizienteren Einfluss auf die hochzervikalen Dysfunktionen und eine schnellere Verbesserung des Asymmetriescores. In the first weeks after birth, the craniocervical region is of particular importance in the development and treatment of infantile postural asymmetries and positional plagiocephaly. This secondary analysis compares how osteopathic treatments affect the passive upper cervical rotation and the degree of postural asymmetry with the effects of physiotherapeutic treatments based on the Vojta concept. A clinical intervention study divided 45 infants with postural asymmetry and positional plagiocephaly into two experimental groups. Group A (OMT-group) received osteopathic treatments once a week over a period of 3–5 weeks. Group B (Vojta-group) received physiotherapy treatment according to the Vojta concept once or twice a week over the same period. 3 months after the first treatment all children got a follow up examination. Changes in the cervical flexion-rotation test and in the asymmetry score were measured during treatment. The findings were statistically analyzed in an intra-group and an inter-group comparison. Both treatment methods significantly improve the degree of asymmetry (OMT-group: p = 3.7E-21; Vojta-group: p = 1.4E-05) and the passive upper-cervical rotation. In the overall rotation (FRT sum), the OMT-group achieved a significant result at the 2nd treatment (T2: p = 0.013) and the Vojta-group at the follow-up appointment (T4: p = 0.035). An isolated analysis of the results of treatments done by osteopaths specialized and experienced in pediatric treatment suggests a significant improvement in the flexion-rotation test (T3: p = 2.8E-03) compared to the Vojta-group. In this analysis both – physiotherapeutic treatments based on the Vojta concept and osteopathic treatments – are effective. The osteopathic treatment has a more efficient influence on the upper-cervical dysfunction and results in a faster improvement of the asymmetry score. [ABSTRACT FROM AUTHOR]

Odontoidectomy may pose some risks for O-C1 and/or C1-C2 instability, with previous authors reporting techniques for endonasal C1-C2 fusion. However, no technique for endonasal O-C1 fusion currently exists. We sought to describe the feasibility of endonasal anterior C1 (AC1) screw placement for endonasal O-C1 fusion. Seven adult cadaveric heads were studied for endonasal placement of 14 C1 screws. Using thin-cut computed tomography (CT)–based "snapshot" neuronavigation assistance, 4 mm x 22 mm screws were placed in the C1 lateral mass using a 0° driver. Post-placement CT scans were obtained to determine site-of-entry measured from C1 anterior tubercle, screw angulation in axial and sagittal planes, and screw proximity to the central canal and foramen transversarium. Average site-of-entry was 16.57 mm lateral, 2.23 mm rostral, and 5.53 mm deep to the anterior-most portion of the C1 ring. Average axial angulation was 19.49° lateral to midline, measured at the C1 level. Average sagittal angulation was 13.22° inferior to the palatal line, measured from the hard palate to the opisthion. Bicortical purchase was achieved in 11 screws (78.6%). Partial breach of the foramen transversarium was observed in 2 screws (14.3%), violation of the O-C1 joint space in 1 (7.1%), and violation of the central canal in 0 (0%). Average minimum screw distances from the unviolated foramen transversaria and central canal were 1.97 mm and 4.04 mm. Navigation-assisted endonasal placement of AC1 screws is feasible. Additional studies should investigate the biomechanical stability of anterior C1 screw-plating systems, with anterior condylar screws as superior fixation point, compared to traditional posterior O-C1 fusion. [ABSTRACT FROM AUTHOR]

Purpose: Occipitocervical junction (OCJ) instability is commonly treated with fixation via open posterior approach. The use of intraoperative navigation allows us to perform occipitocervical fixation via minimally invasive approach. We report a series of patients treated with percutaneous occipitocervical fixation, describing the surgical procedure in detail and discussing the technique. Methods: We prospectively enrolled 8 patients affected by OCJ instability secondary to trauma and rheumatoid arthritis. Traumatic patients were preoperatively evaluated with CT scan and MRI scan if needed. Rheumatoid arthritis group was evaluated with both CT and MR. Patients underwent percutaneous occipitocervical fixation with the assist of intraoperative 3D imaging and navigation. All patients were functionally and radiologically evaluated pre-, at 6 weeks, and at 1 year postoperatively. Results: Percutaneous occipitocervical fixation was successfully performed in all of the patients. 33 screws were placed. 29 (87.88%) were placed without any pedicle breach. In 3 (9.09%) screws we observed a minor; and in 1 (3.03%) screw we observed a major pedicle breach. We did not have any postoperative complications. Conclusions: Described method of occipitocervical fixation is a minimally invasive method that has a similar outcome to the open technique. It requires the experience in open techniques and the assist of intraoperative 3D imaging and navigation to be performed efficiently. [ABSTRACT FROM AUTHOR]

Objective: The purpose of our study was to identify adult trauma patients with an acute C1 burst fracture, evaluate for concomitant transverse atlantal ligament (TAL) injury, and apply the modified Gehweiler and AO spine classification systems to determine the utility of these classification systems in accurately defining C1 trauma. Materials and Methods: Adult trauma patients with an acute C1 fracture were identified retrospectively using Nuance mPower software. The C1 fracture was described based on whether the fracture involved the anterior arch, posterior arch, lateral mass, medial tubercle, and/or transverse process. If follow-up cervical magnetic resonance imaging (MRI) was performed, the presence and location of an associated TAL injury was recorded. The anatomic location of the C1 burst fracture and TAL injury, if present, were compared with the descriptive classification systems outlined by Gehweiler/Dickman (modified) and the AO Spine society. Any additional osseous trauma of the skull base and C1-C2 was also recorded along with relevant clinical history and management. Results: Thirty-nine patients were identified with an acute C1 burst fracture on cervical computed tomography (CT) with seventy-seven percent of patients undergoing follow-up cervical MRI. Observed fracture patterns were divided into five distinct types based on CT findings and further subdivided based on the integrity of the transverse altantal ligament on MRI. TAL tears were observed exclusively in type 3 fractures (anterior and posterior arch fractures) and type 4 fractures (anterior arch, posterior arch, and lateral mass fractures). The modified Gehweiler classification system failed to accurately describe the anatomic location of the C1 fracture in forty-four percent of patients, whereas the AO spine was too broad and failed to accurately describe fracture location in our cohort. Conclusions: The Gehweiler and AO spine classifications demonstrated significant shortcomings in the accurate description of patients with C1 trauma. Whereas the Gehweiler system did not accurately describe the anatomic location of the various C1 fractures, the AO spine system was too broad and failed to radiologically classify fracture location. Moreover, there was a high number of patients with AO spine type B injuries without atlantoaxial translation that nevertheless required C1-C2 fusion for atlantoaxial instability. We suggest the need for an updated classification system that takes into account both the CT (fracture location) and MRI (TAL integrity) appearance of C1 trauma. An updated classification strategy will offer a radiologic standardization of C1 trauma that will aid in future research studies and help optimize patient management. [ABSTRACT FROM AUTHOR]

Background: The surgery on the craniocervical junction is associated with complex techniques that endanger the vertebral artery (VA), especially if there are some anatomical variations present, thereby increasing the risk of vascular injury, particularly during cervical decompression or instrumentation., Case Description: A case of a 60-year-old female with progressive myelopathy and craniocervical junction malformation is presented. Key preoperative imaging findings included basilar invagination, C1 assimilation, and os odontoideum, along with VA anomalies such as a tortuous, hypoplastic left VA arising anomalously from the aortic arch and a right VA with a V2 segment forming a high-riding medial loop into the C2 vertebral body. The surgical procedure was performed through a posterior approach using C-arm fluoroscopy, Doppler ultrasound, and intraoperative neurophysiologic monitoring, which achieved adequate deformity fixation and anterior decompression., Conclusion: Computed tomography angiography is the gold standard for assessing the preoperative VA anatomy at the craniocervical junction. Intraoperative Doppler ultrasound is invaluable because it minimizes the risk of sudden accidental injury to VAs in cases with abnormal anatomies., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)

Background: Spinal arteriovenous fistulae (AVF) located at the craniocervical junction (CCJ) are rare and usually present with hemorrhage. Bleeding is usually attributed to arterial feeders arising from the anterior spinal artery (ASA) and aneurysms located on such feeders. Perimedullary AVFs are typically found on the ventral surface of the spinal cord, which makes them difficult to treat through traditional microsurgical methods. In addition, their unique vessel angioarchitecture frequently precludes safe embolization. We present the first case of a CCJ perimedullary AVF successfully treated using flow diversion., Case Description: A 76-year-old man was brought to the emergency department after suddenly losing consciousness. On further evaluation, infratentorial subarachnoid hemorrhage and a perimedullary AVF at the ventral surface of the spinal cord were identified. The ASA originated from the left V4 segment, providing a single feeder to the lesion associated with a 2 mm aneurysm. After initial antiplatelet loading, 8 hydrophilic polymer-coated flow diverters were deployed to cover the ASA's origin in two sessions, achieving the complete occlusion of the lesion and the aneurysm 5 months later, without evidence of ischemic lesions., Conclusion: CCJ perimedullary AVFs can bleed with devastating consequences. These lesions can be challenging to treat through traditional microsurgical or endovascular techniques. Progressive occlusion with flow diversion is feasible in single-feeder AVFs, theoretically allowing blood flow reorganization to the cervical spinal cord., (Copyright: © 2024 Surgical Neurology International.)

Background: Occipitocervical arthrodesis has a variety of indications to treat craniocervical and atlantoaxial pathologies for which a selective cervical fusion would not provide sufficient stability. Over time, the indications for occipitocervical fusions (OCFs) have evolved, as new technologies and surgical techniques were developed. In this bibliometric analysis, we aim to explore the progression of OCF literature over time, analyzing the trends in publications and citations, publishing countries and authors, keywords, and topics., Methods: The Web of Science database was used for data retrieval on July 3, 2024, with the search "occipitocervical fusion" OR "occipito-cervical fusion" OR "occipitocervical arthrodesis" OR "occipital cervical fusion" OR "occipital cervical arthrodesis" OR ("OCF" AND "spine surgery"). Excel was used to create the citation analysis and publication trend figures, along with the publishing countries and author analysis. The bibliometric software VosViewer was used to generate the keyword co-occurrence network visualizations., Results: Overall, 762 articles were extracted. The number of pertinent publications and citations increased until 2020 before beginning to decrease. We found that Ehlers Danlos syndrome has become a more prevalent topic, as the association between Ehlers Danlos syndrome and craniocervical instability has received further scrutiny. "Dysphagia" continues to be a commonly cited topic, while, conversely, rheumatoid arthritis has decreased in publication frequency, possibly related to advances in medical management and surgical techniques. Overall, the United States, China, and Japan are the top publishing countries., Conclusions: This analysis of OCF literature provides a helpful overview of emerging trends and clinician concerns, especially as seen through the perspective of time., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Posterior inferior cerebellar artery (PICA) dissecting aneurysms are rare and typically present with subarachnoid hemorrhage (SAH) or ischemic symptoms, with a high risk of rebleeding in the acute phase. This case presents an atypical ruptured PICA aneurysm with a hematoma confined to the craniocervical junction and cervical cord, leading to a delayed diagnosis - a 41-year-old male with an atypical presentation of headache and neck pain without neurological deficits. Initial magnetic resonance imaging (MRI) revealed a hematoma extending from the craniocervical junction to the cervical spinal cord without intracranial SAH, leading to misdiagnosis as spinal subdural hematoma. As symptoms worsened, further investigation with MRI and digital subtraction angiography (DSA) uncovered a ruptured PICA-dissecting aneurysm. The patient underwent successful endovascular coil embolization with parent artery occlusion. This case highlights the importance of considering PICA-dissecting aneurysms in craniocervical junctions and spinal cord hemorrhage, even in the absence of typical intracranial SAH. It underscores the need for a high index of suspicion and comprehensive vascular imaging for timely diagnosis and treatment in atypical cases., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Nakamura Memorial Hospital Ethics Committee issued approval 2024082401. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Hiratsuka et al.)

Background: Fibrous dysplasia of the bone is a disease caused by a somatic GNAS mutation that affects craniofacial bones and can have a mass effect on different neurovascular structures. The authors present the first case of primary jugular foramen fibrous dysplasia with occlusion of the transverse and sigmoid sinuses., Objective: A 33-year-old man presented with a history of dizziness and occasional dysphagia over the past year. Magnetic resonance imaging showed a uniform enhanced mass in the left jugular foramen, with complete blockage of the adjacent transverse and sigmoid sinuses. The computed tomography scan revealed a cystic bone lesion of the jugular foramen. The patient underwent a gross-total removal of the tumor through an infratemporal transjugular approach with complete preservation of the lower cranial nerves., Lessons: This first reported case of primary jugular foramen fibrous dysplasia highlights the importance of considering this diagnosis when evaluating jugular fossa lesions. Understanding the anatomy of the infratemporal and jugular fossae, along with proficiency in microsurgical techniques, is essential for removing such tumors while preserving cranial nerve functions and the patient's quality of life. https://thejns.org/doi/10.3171/CASE24396.

A comparative study of the morphology of suboccipital cavernous sinus (SCS) using MRI and cast specimens was performed. The present retrospective study analysed the craniocervical magnetic resonance venography (MRV) imaging data of 61 patients. Three-dimensional reconstruction was performed using Mimics 19.0. The SCS left-right diameter(d1), distance from the midline (d2), supero-inferior diameter(d3), anteroposterior diameter (d4), distance from posterior diameter to skin (d5), and diameter of the SCS at different parts (d6-d8) were measured. Comparison between MRV images and cast specimens, the SCS, marginal sinus, anterior condylar vein, and vertebral artery venous plexus were symmetrical and could be bilaterally displayed, whereas the presence of extra condylar vein and posterior condylar vein exhibited different types. The adjacency between the SCS and its communicating vessels and changes in its communicating vessels corresponded well with the MRV images and cast specimens. Many types of the presence of left and right lateral condylar and posterior condylar veins were found in the cast specimens, which could be divided into the bilateral presence of posterior condylar and lateral condylar veins, unilateral presence of posterior condylar veins, and unilateral presence of lateral condylar vein. A total of 61 cases analysed using MRV images revealed the bilateral presence of posterior condylar and lateral condylar veins (77.1 %), the unilateral presence of posterior condylar vein (18.0 %), and the unilateral presence of lateral condylar vein (9.8 %), of which the bilateral presence of posterior condylar and lateral condylar veins accounted for the largest proportion. MRV images and cast specimens of the SCS showed its normal morphological structure and adjacency, thus providing accurate and complete Three-dimensional imaging anatomical data of the SCS and its communicating vascular structures. This study enriches the Chinese SCS imaging anatomy data and may be valuable in clinical practice. [ABSTRACT FROM AUTHOR]

Context: Atlanto-occipital dissociation is a highly lethal ligamentous injury at the craniocervical junction (CCJ). Previous studies have described rare cases of milder forms of atlanto-occipital injury (AOI) which might be managed nonoperatively, but there is a paucity of literature on this subject. Aims: We retrospectively reviewed our institutional experience to characterize the injury patterns, treatments, and clinical courses of patients with unilateral AOI. Methods: We included patients with radiographic evidence of unilateral occipitocervical joint capsular disruption, distraction, or edema ± injury of the apical ligament, tectorial membrane, anterior atlanto-occipital membrane, posterior atlanto-occipital membrane, alar ligaments, or cruciate ligament. The long-term outcomes were gathered from medical records, and six patients were available for Neck Disability Index via phone call at the time of the study. Results: Eight patients were included in the study. The mean age was 45.1 years ± 26.5. Causes of trauma included motor vehicle collision for five patients (5/8, 62.5%), falls for two (2/8, 25), and assault for one (1/8, 12.5%). All patients had a widened condyle-C1 interval >2 mm. Three patients underwent occipitocervical fusion, one patient underwent atlantoaxial fusion, and another received subaxial fusions for other injuries. Three patients underwent no surgical intervention. All patients were seen at least once as an outpatient following hospital discharge. There were no delayed neurologic injuries or deaths. Conclusions: We propose that ligamentous injury at the CCJ functions more as a spectrum rather than dichotomous diagnosis, of which a subset can likely be safely managed nonoperatively. [ABSTRACT FROM AUTHOR]

Os odontoideum (OO) is a rare craniocervical anomaly that is characterized by a round ossicle separated from the C2 vertebral body. With a controversial etiology and unknown prevalence in the population, OO may be asymptomatic or present in patients with myelopathic and neurological symptoms. In this literature review, we aimed to investigate epidemiology, embryology, pathophysiology, clinical presentation, and the role of diagnostic radiography in OO. By providing an overview of different management strategies, surgical complications, and postoperative considerations for OO, our findings may guide neurosurgeons in providing proper diagnosis and treatment for OO patients. A literature review was conducted using the PubMed, EMBASE, and Scopus databases. A search using the query "os odontoideum" yielded 4319 results, of which 112 articles were chosen and analyzed for insights on factors such as etiology, clinical presentation, and management of OO. The presentation of OO varies widely from asymptomatic cases to severe neurological deficits. Asymptomatic patients can be managed either conservatively or surgically, while symptomatic patients should undergo operative stabilization. Although multiple studies show different techniques for surgical management involving posterior fusion, the diversity of such cases illustrates how treatment must be tailored to the individual patient to prevent complications. Conflicting studies and the paucity of current literature on OO highlight poor comprehension of the condition. Further understanding of the natural history of OO is critical to form evidence-based guidelines for the management of OO patients. More large-center studies are thus needed to promote accurate management of OO patients with optimal outcomes. [ABSTRACT FROM AUTHOR]

In the diagnosis of an intracranial dural arteriovenous fistula (DAVF), arterial spin labeling (ASL), a sequence of magnetic resonance imaging (MRI) to depict high-blood-flow intracranial lesions, has been reported as a useful and noninvasive tool, not only to predict the presence of cortical venous drainage and draining veins, but also to confirm persistent obliteration after treatment. However, such utility of ASL has not been reported in DAVF of the craniocervical junction (CCJDAVF) because of the rarity of this disease and uncertainty in the acquisition of precise images.We report a case of CCJDAVF presenting with myelopathy. Preoperative ASL images showed an abnormal high-intensity signal in the craniocervical junction, consistent with the anterior spinal vein and draining veins, which were also identified by digital subtraction angiography. After successful surgical treatment for the disease, MRI and 4-dimensional computed tomography angiography (4DCTA) confirmed complete disappearance of CCJDAVF. The ASL images also showed no abnormal intensity signal. The patient was followed-up using ASL, and no recurrence of high-intensity signal was observed.As repetitive image examination is mandatory in the follow-up of a patient with DAVF to exclude recurrence, ASL is highly beneficial because of the unnecessity of an exogenous contrast medium and high credibility to depict the disease. The craniocervical junction may be out of the field of view in routine MRI. Special attention must be paid to setting the field of view and post labeling delay (PLD) to obtain precise images of ASL in CCJDAVF.

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

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

Background: Transoral odontoidectomy followed by occipitocervical fixation is an accepted technique for the treatment of ventral compression at the craniocervical junction (CVJ). It is thought that surgical experience has stagnated owing to both the development of endonasal approaches, and improvement in rheumatoid pharmaceutical therapies. Accurate knowledge on whether the declining experience affects rates of intra-operative and peri-operative complications is essential. Herein, we present an illustrated technical note and case series based on our 10-year surgical experience. Methods: Technical pearls for the transoral ondontoidectomy approach are discussed and illustrated. A retrospective analysis of patients aged 18 years or older who underwent the procedure over the last 10 years was conducted. Both intra and post-operative complications were recorded and compared to historical controls. Results: Ten patients were identified with an average of 60 years of age and follow-up time of 15 months. Indications for surgery included rheumatoid (10%), degenerative (30%) and pseudogout cervical pannus (10%), basilar invagination (30%), and malignancy (20%). Average length of stay was 19.4 days. Two patients suffered from intra-operative CSF leaks while 8 patients suffered from post-operative medical complications including the need for tracheostomy for 4 individuals. Rate of improved neurological outcomes was 60% while 20% of patients were stable at last clinic follow-up. Conclusion: In this study, we describe our technique to obtain exposure and perform decompression of CVJ ventral pathologies via the transoral route. The intra and post-operative complications we report represent the common pitfalls. Highly specialized and comprehensive patient care remains essential as post-operative complication rates remain high.

Abstract Background Several central nervous system diseases are associated with disturbed cerebrospinal fluid (CSF) flow patterns and have typically been characterized in vivo by phase-contrast magnetic resonance imaging (MRI). This technique is, however, limited by its applicability in space and time. Phase-contrast MRI has yet to be compared directly with CSF tracer enhanced imaging, which can be considered gold standard for assessing long-term CSF flow dynamics within the intracranial compartment. Methods Here, we studied patients with various CSF disorders and compared MRI biomarkers of CSF space anatomy and phase-contrast MRI at level of the aqueduct and cranio-cervical junction with dynamic intrathecal contrast-enhanced MRI using the contrast agent gadobutrol as CSF tracer. Tracer enrichment of cerebral ventricles was graded 0–4 by visual assessment. An intracranial pressure (ICP) score was used as surrogate marker of intracranial compliance. Results The study included 94 patients and disclosed marked variation of CSF flow measures across disease categories. The grade of supra-aqueductal reflux of tracer varied, with strong reflux (grades 3–4) in half of patients. Ventricular tracer reflux correlated with stroke volume and aqueductal CSF pressure gradient. CSF flow in the cerebral aqueduct was retrograde (from 4th to 3rd ventricle) in one third of patients, with estimated CSF net flow volume about 1.0 L/24 h. In the cranio-cervical junction, net flow was cranially directed in 78% patients, with estimated CSF net flow volume about 4.7 L/24 h. Conclusions The present observations provide in vivo quantitative evidence for substantial variation in direction and magnitude of CSF flow, with re-direction of aqueductal flow in communicating hydrocephalus, and significant extra-cranial CSF production. The grading of ventricular reflux of tracer shows promise as a clinical useful method to assess CSF flow pattern disturbances in patients. Graphic abstract

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.

Objective: Spinal reconstruction after resection of invasive craniocervical junction malignancies is fraught with technical and management considerations as well as a paucity of data in the existing literature. In this study, we describe our experience with craniocervical junction malignancies, especially the influence of radiation on the need for revision spinal instrumentation. Methods: We performed a retrospective chart review of all patients who underwent occipitocervical fixation between 2011 and 2019 at The University of Texas MD Anderson Cancer Center. Results: Twenty-five patients had primary malignancies and 12 (30%) had metastatic tumors. Thirteen (33%) underwent a staged resection in multiple operations during their hospital stay. Tumor resection was performed in 19 patients (48%), while only stabilization was performed in 21 patients (52%). Nine patients (23%) underwent expanded endoscopic transclival approaches for tumor resection, 10 patients (25%) an extreme lateral approach, and 2 patients (5%) an anterior open approach. Eleven patients underwent early postoperative radiation therapy (within 3 months) and 8 underwent delayed radiation therapy (between 3 months and 1 year in 7 patients). The revision rate was 8%, with a median time to revision surgery of 42 months. The administration and timing of adjuvant radiation therapy relative to surgery had no significant effect on the need for instrumentation revision on log-rank and Cox regression analyses (p < 0.05). Conclusion: Revision surgery was needed infrequently in our patients. Postoperative radiation therapy was not associated with hardware failure, indicating that the timing of radiation therapy should be dictated by the diagnosis and can be initiated postoperatively without delay. [ABSTRACT FROM AUTHOR]

A neonatal Dutch Warmblood colt was evaluated for inability to stand, incoordination and intention tremor. Despite partial improvement in clinical signs during the first 4 days of hospitalization, neurological deficits remained. Magnetic resonance imaging identified a unilateral infratentorial arachnoid cyst‐like lesion with ipsilateral compression and displacement of the cerebellar hemisphere, absent corpus collosum, polymicrogyria, suspect leukoencephalopathy, and noncompressive occipitoatlantal malformation. Improvement in clinical signs during the first 6 months of life suggests that horses can survive with complex congenital neurological malformations, but prognosis for athletic performance is poor. The accessibility of magnetic resonance imaging should improve the diagnostic accuracy of central nervous system disorders in neonatal foals in which congenital malformations are suspected. Euthanasia often is elected in foals with suspected congenital central nervous system disorders because of poor prognosis for athletic performance, limiting understanding of clinical progression in these cases. [ABSTRACT FROM AUTHOR]

Background and Purpose: Craniocervical dissociation is a rare and life-threatening injury that results from a significant hyperflexion-hyperextension force. Occult craniocervical dissociation is defined as an unstable craniocervical injury in the absence of atlanto-occipital joint space widening or other skull base line abnormality. The early and accurate diagnosis of craniocervical dissociation is crucial since the early diagnosis and subsequent stabilization with occipital-cervical fusion has been shown to reduce neurologic morbidity and mortality. Several normative skull base lines have been developed to predict craniocervical dissociation. The purpose of our study was to measure the atlanto-occipital joint space and four other common skull base lines in patients who underwent occipital-cervical fusion for post-traumatic craniocervical instability.Materials and Methods: Patients who underwent occipital-cervical fusion for craniocervical injury were identified retrospectively using a keyword search of radiology reports using Nuance mPower software. The cervical CT and MRI exams for these patients were reviewed and the atlanto-occipital joint space, Powers ratio, Wackenheim line, posterior axial line, and basion dens interval were measured. Detailed descriptions of craniocervical ligament injuries on MRI were recorded along with patient demographic information, clinical history, management, and outcome.Results: Nine adult patients who underwent occipital-cervical fusion for an acute craniocervical injury were identified. Six patients demonstrated an atlanto-occipital joint space measuring 2 mm or less on cervical spine CT with no additional abnormality in the Powers ratio, Wackenheim line, posterior axial line, or basion-dens interval. Three patients demonstrated widening of the atlanto-occipital joint space with two patients demonstrating an abnormality in at least two additional skull base lines. Clinical outcomes were variable with nearly half of the patients demonstrating persistent neurologic deficits, including one quadriplegic patient.Conclusions: A normal atlanto-occipital joint space and skull base line measurements on cervical CT demonstrated a low predictive value for detecting unstable craniocervical injuries. Occult craniocervical dissociation was present in two-thirds of patients who underwent occipital cervical fusion for acute, craniocervical trauma. A high clinical and radiologic index of suspicion for craniocervical trauma with subsequent follow-up cervical MRI to directly evaluate ligamentous integrity is necessary to accurately diagnose and triage patients with high velocity trauma. [ABSTRACT FROM AUTHOR]

Purpose: Atlas-duplication is an exceedingly rare dysplasia of the craniocervical junction. To the best of our knowledge, only two cases of atlas-duplication have been reported and these were associated with complete anterior rachischisis and os odontoideum. We aimed to report a case of isolated atlas-duplication of incidental finding and without attributable symptoms which makes it unique. Methods: Following a normal coronarography for a suspected myocardial infarction, a 60-year-old-man with no significant medical history developed a transient ischemic attack that justified brain computed-tomography angiography. Results: There was no evidence for cerebral ischemic lesion, intracranial occlusion or significant artery disease. Bone analysis revealed eight cervical vertebral segments with an additional vertebral level located between the occiput and the atlas. This vertebra presented all the morphological characteristics of an atlas vertebra except for hypoplasia of the left transverse process. An incomplete anterior rachischisis was associated, and there was no other abnormality of craniocervical junction. The clinical examination revealed no neck pain, no limitation of joint amplitude and no neurological deficit. Apart from preventive treatment of ischemic stroke, no orthopedic or surgical treatment was undertaken. After 1.5 years of radiological monitoring, the patient remains symptom-free. Conclusions: Atlas-duplication is an exceedingly rare dysplasia of the craniocervical junction that may be found isolated and incidentally. If this variation does not necessarily warrant specific treatment, brain CT angiography is recommended to detect anatomical variations of the vertebral arteries. [ABSTRACT FROM AUTHOR]