C2 Nerve Root Preservation During Posterior Fixation for Instability Secondary to Congenital Craniovertebral Junction Anomalies: Feasibility Factors and Related Outcomes.

OBJECTIVE: Patients with instability because of congenital craniovertebral anomalies often have complex C1-C2 osseovascular anomalies. C2 nerve root sacrifice has been described to address such difficult anatomy during posterior C1-C2 fixation and has its own downsides. Its preservation as a recent alternative poses greater surgical challenge, and the considerations differ from other causes of craniovertebral junctional instability; the pertaining outcomes have been scarcely studied. The objective of this study was to prospectively determine the feasibility and outcomes related to C2 nerve root preservation in patients with congenital atlantoaxial dislocation (CAAD) after posterior C1-C2 fixation. METHODS: In this observational study, 63 patients (126 nerve roots) with CAAD after posterior fixation were prospectively assessed. Underlying osseovascular anomalies affecting the feasibility of C2 nerve root preservation, and C2 nerve-related dysfunction at 12 months follow-up were analyzed. RESULTS: The overall C2 nerve root preservation rate was 89.7%. Even in the presence of extreme joint obliquity/spondyloptosis and anomalous vertebral artery, it was feasible in about three fourths. After preservation, 28.3% patients developed new-onset C2 nerve root dysfunction: neuralgia in 2, dysesthesia in 6, and hypoesthesia/paresthesia in 9. The symptoms were not disabling in most patients. CONCLUSIONS: In most patients with CAAD, C2 nerve root preservation is feasible despite an aberrant bony and vascular anatomy. A few patients after nerve root preservation develop related symptoms that are conservatively manageable, with no significant adverse consequences. Given the controversy in the literature on C2 nerve sacrifice-related outcomes, we favor an attempt at C2 nerve root preservation.

Tumor vertebral primario de axis: un desafío clínico y quirúrgico / Primary vertebral tumor of axis: A clinical and surgical challenge

Presentamos el caso de un varón de 17 años que acude a urgencias por cervicalgia de 6 semanas de evolución, sin déficit neurológico. La tomografía y resonancia magnética mostraban una masa dependiente de C2 con infiltración de partes blandas e invasión del canal medular, sin signos de mielopatía. Se realizó biopsia guiada con tomografía. Esperando al resultado, el paciente desarrolló una tetraparesia aguda con incompetencia de esfínteres. Una nueva resonancia magnética mostró un aumento de la masa ósea tumoral con extensión epidural, mielopatía y nuevas lesiones vertebrales. Se realizó una resección posterior y descompresión medular de urgencia, con fijación occipito-cervical. La recuperación postoperatoria fue completa. La inmunohistoquímica reveló un sarcoma de Ewing. Se inició quimioterapia con respuesta parcial. El sarcoma de Ewing primario atlantoaxial es una entidad excepcional con mal pronóstico. Su tratamiento es multidisciplinar, incluyendo una resección total si es posible, lo cual supone un reto para el cirujano (AU)

We report the case of a 17-year-old male presented to the emergency department with a six weeks history of neck pain and no neurogical deficit. Computed tomography and magnetic resonance imaging revealed an expansile lesion in the axis, with soft tissue and spinal cavity invasion, without mielopathy signs. Tomography-guided mass biopsy was taken. Waiting for histopathologic results, the pacient developed acute tetraparesis and sphincter incompetence. Magnetic resonance revealed that the bone mass had grown with epidural compromise, mielopathy and new vertebral lesions. Medular decompression with laminectomy, excision of the posterior elements of axis with the involved soft tissue mass and occipito-cervical fixation was performed. Neurological recovery was complete. Inmunochemistry revealed an Ewing Sarcoma. Chemoteraphy treatment was given, with partial response. Primary Ewing sarcoma of atlas-axis is a rare entity with poor prognosis. Multidisciplinary approach treatment is needed, with a total surgical resection if it is possible, a real challenge for the surgeon (AU)

Upper Cervical Trauma.

Craniocervical injuries (CCJs) account for 10% to 30% of all cervical spine trauma. An increasing number of patients are surviving these injuries due to advancements in automobile technology, resuscitation techniques, and diagnostic modalities. The leading injury mechanisms are motor vehicle crashes, falls from height, and sports-related events. Current treatment with urgent rigid posterior fixation of the occiput to the cervical spine has resulted in a substantial reduction in management delays expedites treatment of CCJ injuries. Within CCJ injuries, there is a spectrum of instability, ranging from isolated nondisplaced occipital condyle fractures treated nonoperatively to highly unstable injuries with severely distracted craniocervical dissociation. Despite the evolution of understanding and improvement in the management of cases regarding catastrophic failure to diagnose, subsequent neurologic deterioration still occurs even in experienced trauma centers. The purpose of this article is to review the injuries that occur at the CCJ with the accompanying anatomy, presentation, imaging, classification, management, and outcomes.

Proposal of New Radiological Classification and Treatment Strategy for Transverse Fractures of the C2 Axis Body.

OBJECTIVE: To investigate the characteristics of transverse fractures of the C2 axis body diagnosed on sagittal computed tomography (CT) and to propose new classification and appropriate treatment strategies. METHODS: A retrospective study was performed by enrolling 49 patients (26 men and 23 women) with transverse fractures of the C2 axis body who were treated at four national trauma centers of tertiary university hospitals from January 2000 to December 2017. The mean age of the patients was 60.8 years (ranging from 21 to 90 years). We classified 49 transverse fractures of the C2 body into three types based on fracture trajectories involving superior articular facet (SAF) and lateral cortex (LC) of the C2 body on coronal CT as follows: Type 1, involvement of C2 SAF on both sides; Type 2, unilateral involvement of C2 SAF on one side and LC on the other side; Type 3, involvement of LC on both sides. The characteristics, treatment methods, and results of 49 transverse fractures of the C2 body were analyzed. Mean follow-up was 12.6 months (ranging from 12 to 26 months). RESULTS: Twenty-six (53.1%) patients were Type 1, 21 (42.9%) were Type 2, and 2 (4.0%) were Type 3. Correlation coefficients for intra-observer and inter-observer reliabilities of classification were 0.723 and 0.598 (both, P < 0.001), respectively. About 40.8% (7 Type 1 and 13 Type 2) of the patients had fracture displacement >3 mm; Incidence of fracture displacement >3 mm was higher in Type 2 than Type 1 (61.9% vs 26.9%, P < 0.05). About 79.6% (20 Type 1, 17 Type 2 and 2 Type 3) of the patients were treated conservatively, and 20.4% (6 Type 1 and 4 Type 2) underwent surgery. At last follow-up, 47 out of 49 patients achieved fusion; overall fusion rate was 95.9%. All conservatively treated Type 1 and Type 3 patients achieved fusion. Out of 17 conservatively treated Type 2 patients, 15 achieved fusion but two developed nonunion; however, two nonunion patients opted not to undergo surgery. Subgroup analysis showed that Philadelphia brace caused nonunion significantly in fracture displacement >3 mm compared to Minerva brace/Halovest (100% vs 0%, P < 0.05). All surgically treated Type 1 and 2 patients achieved fusion. In terms of clinical outcomes, neck pain visual analog scale and neck disability index were significantly improved (both, P < 0.01). According to Odom's criteria, 93.9% (46/49) of the patients achieved satisfactory outcomes. No major complications occurred. CONCLUSIONS: The majority of transverse fractures of C2 body can be treated conservatively. However, surgery or rigid Minerva brace/Halovest should be considered for Type 2 transverse fractures of the C2 body with fracture displacement >3 mm.

Biologic Agents Preserve the C-2 Pedicle in Patients with Rheumatoid Arthritis: A Comparative Imaging Study Using Three-Dimensional Computed Tomography.

OBJECTIVE: To investigate whether biologic agents (BAs) reduce a narrow C-2 pedicle screw trajectory, which is often a key stabilizer in surgical treatment, in patients with rheumatoid arthritis (RA). METHODS: A total of 100 patients with RA treated with and without BAs (BA [+] group [n = 50] and BA [-] group [n = 50]), respectively, were included in the present study. Computed tomography (CT) images of their cervical spine, including C-2, were analyzed. The maximum screw diameter at C-2 that could be inserted without breaching the cortex, measured on 3-dimensional images using a CT-based navigation system, was compared between the groups with and without BA administration. Furthermore, the destruction of the atlantoaxial joint was examined using CT images. The risk factors for a narrow C-2 pedicle were elucidated among the patients treated with BAs. RESULTS: The pedicle in the BA (+) group had a significantly larger C-2 maximum screw diameter than the BA (-) group (6.00 mm vs. 5.13 mm, P < 0.001), with less destruction of the atlantoaxial joint. Among the BA (+) group, a longer period until the initial administration of BAs and RA disease duration were associated with a narrow C-2 pedicle. CONCLUSIONS: This study suggests that BAs can maintain the trajectory for C-2 pedicle screws, which acts as a key stabilizer in surgical management for the rheumatoid cervical spine, by halting the destruction of the atlantoaxial joint. Early introduction of BAs can be especially important to prevent the narrowing of the C-2 pedicle.

C2 Pedicle Sclerosis Grading, More Than Diameter, Predicts Surgeons' Preoperative Assessment of Safe Screw Placement: A Novel Classification System.

BACKGROUND: The preoperative assessment of C2 morphology is important for safe instrumentation. Sclerotic changes are often seen in C2 pedicles. Evaluating the diameter measurements solely might not accurately assess the safety of screw insertion. We have proposed a novel grading system of the C2 pedicle that includes sclerosis and evaluated the predictive value of this grading system with the surgeon's safety evaluation. METHODS: We reviewed and measured the dimensional values in 220 cervical computed tomography angiograms. Additionally, we used a grading system that divides the findings into 5 grades according to the width measurement and degree of sclerosis in the C2 pedicle. Two spine surgeons independently classified the pedicles as follows: safe (minimal risk of pedicle violation), caution needed (caution to minimize pedicle violation), or dangerous (a high risk of pedicle violation). Finally, we compared the measurements and the surgeons' safety assessments. RESULTS: A total of 411 pedicles of 203 patients (mean age, 69.5 years; 49.5% women) were included. Of the 411 C2 pedicles, 170 were classified as high risk by ≥1 surgeon. Between the dimensional measurements and grading system, the sclerotic grade showed the best predictive value. CONCLUSIONS: We have introduced a novel tool to evaluate the safety of C2 pedicle screw placement. Our results suggest that our pedicle width-sclerosis grading system is reproducible and predicts the surgeon's assessment of safe screw placement better than C2 pedicle diametrical measurements alone.

4-Point C2 Fixation for Occipitocervical Fusion: Technical Case Report.

BACKGROUND: Occipitocervical fusion is an important surgical procedure to treat instability of the upper cervical and craniocervical junction. Fixation to the dense cortical bone of the occiput, contemporaneously typically accomplished with a plate and screws, is known to be strong and durable, but there are many competing methods used to secure an adequate number of fixation points of sufficient strength at the cervical end. Extension of hardware to the midcervical region to acquire additional fixation points, however, results in loss of subaxial motion segments and additional potential morbidity. The C2 vertebra is unique in that its morphology and dimensions permit fixation with longer screws than are typically possible to place in the midcervical lateral masses. Translaminar and pars screw techniques, both commonly used to achieve C2 fixation, are not mutually exclusive, as their respective trajectories are considerably different and engage different portions of the bony anatomy. METHODS: We describe a novel, 4-point C2 fixation technique for OC fusion that may avoid the need to extend fusion to the subaxial spine. RESULTS: This technical note illustrates how 4-point C2 fixation can be employed in occiptocervical fusion. CONCLUSIONS: 4-point fixation of C2 combining translaminar and pars screw placement is technically feasible and may be a suitable strategy to spare subaxial motion segments in OC fusion procedures. Futher investigation may establish its applicability to additional surgical procedures.

Chondrosarcoma in the body of the C2 vertebral axis.

Chondrosarcoma, a malignant bone tumor, is rarely encountered in the cervical spine. This article describes a patient whose neck pain and dysphagia were caused by an expansive, destructive lesion with calcification that was located in the body of the axis (C2 vertebra), the first time a chondrosarcoma has been reported in this location.

Rare anatomic variation on a 10,000-year-old South American skeleton: the case of Iuzu, Toca dos Coqueiros, Piauí State, Brazil.

PURPOSE: A skeleton named Iuzu has been unearthed from an exceptional middle Holocene burial in Toca dos Coqueiros site, in Serra da Capivara National Park (UNESCO World Heritage Site, Piauí State, Brazil). During a bioarchaeological analysis of its remains, we discovered that Iuzu was suffering from rare vertebral malformations. A double foramen transversaria, the agenesis of a foramen on the atlas and the hypoplasia of the transverse process of the axis have been highlighted. We aimed to deduce the clinical consequences of the malformation on the patient's health. METHODS: We proceeded to macroscopic observation and radiography of the bones, then we search for other examples of such a pathology in archaeological litterature. RESULT: The malformation caused vascular insufficiency that may have led to neurological lesions leading to various pains and troubles. The very rare malformations Iuzu presented have not been found on a paleoindian skeleton from South America so far. CONCLUSION: This case allowed us to examine the conditions of selection of individuals buried in southern Piauí during the Middle Holocene, during which time this rite does not seem to predominate.

Anatomo-radiological importance and the incidence of os odontoideum in Turkish subjects: a retrospective study.

PURPOSE: Os odontoideum is a rare anatomical and morphological variation of the odontoid process and associated with a range of symptoms such as spinal cord and vertebral artery injuries. This study aimed to evaluate the frequency of os odontoideum in Turkish cases by sagittal/coronal cervical magnetic resonance imaging (MRI) and computed tomography (CT) and analyze the relationship with age, gender and related symptoms. METHODS: The incidence of os odontoideum was retrospectively diagnosed by sagittal/coronal cervical CT and MRI out of 16,122 subjects aged 20-70 years (mean 46) in the period between 2014 and 2018. The relationship of os odontoideum with age, gender, and symptoms was recorded. RESULTS: The statistical analysis of the study was performed by the χ² test and two-way mixed ANOVA. Os odontoideum was detected in 18 (0.11%) (11 males; 7 females) out of 16,122 patients. The mean age was 47.5 ± 1.4 years in the females and 43.5 ± 2.5 years in the males (p < 0.05). 6 odontoideum were detected out of 6467 (3756 males, mean 48 ± 0.7, 2711 females, mean 46 ± 1.2) subjects by CT and 12 odontoideum were detected out of 9655 patients (5607 males and 4048 females) by MRI. CONCLUSION: Neck pain was the most frequent symptom. The prevalence of os odontoideum especially round type is more frequent in older male patients over 40 years old with head and neck pain or atlantoaxial instability, and is less common in Turkish subjects when compared to various ethnic groups.

Os Odontoideum in Children.

Os odontoideum is a rare entity of the second cervical vertebra, characterized by a circumferentially corticated ossicle separated from the body of C2. The ossicle is a distinct entity from an odontoid fracture or a persistent ossiculum terminale. The diagnosis may be made incidentally on imaging obtained for the workup of neck pain or neurologic signs and symptoms. Diagnosis usually can be made with plain radiographs. MRI and CT can assess spinal cord integrity and C1-C2 instability. The etiology of os odontoideum is a topic of debate, with investigative studies supporting both congenital and traumatic origins. A wide clinical range of symptoms exists. Symptoms may present as nondescript pain or include occipital-cervical pain, myelopathy, or vertebrobasilar ischemia. Asymptomatic cases without evidence of radiologic instability are typically managed with periodic observation and serial imaging. The presence of atlantoaxial instability or neurological dysfunction necessitates surgical intervention with instrumentation and fusion for stability.

A Novel Instrumentation Approach in a Pediatric Patient with Atlanto-Occipital Dislocation and Cervical Fracture: Case Report.

BACKGROUND: Although instrumented stabilization of pediatric atlanto-occipital dislocation (AOD) has been described in the literature, there is little evidence regarding instrumentation techniques in pediatric patients presenting with both AOD and a cervical fracture. We present a case of a 2-year-old male involved in a motor vehicle collision with an unstable C2 fracture and AOD, treated with an occiput-C4 posterior arthrodesis using a rod, crosslink, and cable construct. CASE DESCRIPTION: This patient suffered a type III C2 fracture and AOD with 4 mm craniocaudal and 3 mm anterior displacement. In the operating room, 2 cobalt chrome connecting rods (3.5 mm) were connected to 1 another with crosslinks at C2 and C4. These were affixed with suboccipital and sublaminar cables at C1, C2, and C4. At 14 months postoperatively, his spine is clinically and radiographically stable. He has spontaneous movement in all 4 extremities, and remains in a persistent vegetative state because of his underlying central nervous system injury. CONCLUSIONS: Although there is a breadth of literature investigating instrumentation approaches to pediatric AOD, there is minimal evidence on outcomes of patients presenting with both AOD and cervical fracture. The technique we describe has proven safe and effective for this patient.

Mobilization of the Vertebral Artery-Surgical Option for C2 Screw Fixation in Cases With "High Riding" Vertebral Artery.

BACKGROUND: Mobilization of intraosseous course of vertebral artery for safe screw insertion into pedicle of axis in cases with high riding vertebral artery is discussed. OBJECTIVE: To show drilling, exposure, and mobilization of the "high-riding" vertebral artery loop during its course in relationship with superior facet/pedicle of C2 vertebra can provide safety to techniques of atlantoaxial fixation that involve insertion of screws in the pars/pedicle/facet of C2. METHODS: During the period June 2016 to April 2018, 15 patients operated for atlantoaxial stabilization underwent vertebral artery exposure and mobilization using the technique discussed. The ages of the patients ranged from 6 to 48 yrs. Four patients had mobile and reducible atlantoaxial dislocation. Seven patients had basilar invagination. Four patients had os-odontoideum. Gentle and precise drilling of bone that covers the "high-riding" vertebral artery can lead to its safe and wide surgical exposure. The arterial loop can then be either completely or partially mobilized out of the vertebral artery groove such that C2 screw insertion can be conducted under direct surgical visualization of the vertebral artery. RESULTS: During the average follow-up period of 14 mo, there have been no complications related to the surgical procedure, metal implant, or vertebral artery. CONCLUSION: Vertebral artery exposure and mobilization is rather simple and safe procedure and is remarkably effective in providing a space for C2 screw implantation.

Os Odontoideum: Analysis of 190 Surgically Treated Cases.

OBJECTIVE: The aim of this study was to report an experience with 190 cases of os odontoideum over 20 years. The management outcome following atlantoaxial fixation was analyzed. METHODS: From January 2000 to September 2018, 190 patients with os odontoideum were surgically treated. There were 113 male patients and 77 female patients; average age was 24 years (range, 2-68 years).The patients were divided into 3 groups depending on the nature of atlantoaxial dislocation (group 1, mobile and partially or completely reducible atlantoaxial dislocation; group 2, fixed or irreducible atlantoaxial dislocation; group 3, presence of basilar invagination). There were 65 pediatric patients (<18 years old). All patients underwent atlantoaxial joint manipulation and lateral mass plate and screw fixation. The goal of surgery was segmental atlantoaxial arthrodesis. No transoral or posterior foramen magnum bone decompression was done. Occipital bone was not included in the fixation construct. RESULTS: On direct bone handling and observation, atlantoaxial joint pathologic hyperactivity related instability was identified in all patients. Atlantoaxial segmental stabilization resulted in clinical symptomatic and neurologic improvement in 100% of patients. CONCLUSIONS: Os odontoideum signifies chronic or long-standing atlantoaxial instability. Segmental atlantoaxial fixation is a reliable form of surgical treatment. Bone decompression is not necessary. Inclusion of occipital bone and subaxial vertebrae in the fixation construct is not necessary.

Os odontoideum: diagnosis and role of imaging.

OBJECTIVE: To elucidate the imaging manifestations of os odontoideum, establish the diagnosis and guide surgical therapy. METHODS: Clinical and imaging data, including X-ray, CT and MR of 24 patients with os odontoideum, were retrieved and reviewed retrospectively. RESULTS: Os odontoideum with intact cortex was divided into round, conical and blunt tooth types. Four cases of orthotopic and 20 cases of dystopic os odontoideum were included. There was anterior displacement of the base of the dens in six cases, posterior displacement in nine cases and no displacement in nine cases. A widening of anterior atlanto-axial space was shown in 14 patients with varying degrees. Thickening of the soft tissue posterior to the dens was observed in 19 patients, spinal canal stenosis in 21 patients, cervical myelopathy in 10 patients and craniocervical junction malformation in 9 patients. Posterior C1-C2 pedicle screw fixation and fusion was performed in 12 patients and 4 patients underwent posterior occipito-cervical fixation and fusion. CONCLUSION: Radiographically, os odontoideum is defined as an independent ossicle of variable size with smooth circumferential cortical margins separated from the axis. Imaging can be used to assess atlanto-axial instability, associated normal or abnormal anatomical structures and guide surgical therapy.

The Rare Anomaly That Can Be Confused With Fracture: Os Odontoideum.

Os odontoideum is a rare anomaly of the second cervical vertebra. The odontoid process is separated by a wide gap from the vertebral body in this anomaly. It can be associated with atlantoaxial instability.

Surgical Fixation Using Screw-Rod Construct Instrumentation for Upper Cervical Instability in Pediatric Down Syndrome Patients.

STUDY DESIGN: Retrospective case series. OBJECTIVES: To describe the indications and outcomes of cervical fixation using modern instrumentation in a case series of pediatric Down syndrome (DS) patients. SUMMARY OF BACKGROUND DATA: Cervical instability is the major cervical spine concern in children with DS. Although fixation techniques have advanced over the past quarter-century, the outcome of fixation with modern instrumentation for upper cervical instability in DS patients has not been thoroughly investigated. METHODS: We searched the orthopedic database at our institution for patients with a diagnosis of DS who had undergone a cervical spine fusion between 2006 and 2017. Patient demographics, diagnoses, surgical indications, surgical details, and complications were recorded. Preoperative imaging was reviewed to determine atlanto-dens intervals and spinal cord signal changes. Postoperative radiographs or CT scans were reviewed to determine union. RESULTS: Twelve DS patients met our inclusion criteria. The mean age at surgery was 9.3 years (range 3.8-18.8 years). Patients with secondary causes of instability included 7 patients with os odontoideum and 1 patient with a pars fracture. Three patients (25%) were identified on asymptomatic screening, with none of these having cord signal changes on magnetic resonance imaging (MRI). Modern implants (screws, plates, cages) were used in every patient in our series. The mean number of levels fused was 1.9 (range 1-5). The overall complication rate was 41.7% (5/12). Four patients required repeat surgery for nonunion. All patients with adequate radiographic follow-up demonstrated union (11/11, 100%). One patient was lost to follow-up. CONCLUSIONS: Fixation for cervical instability is a critical component of the management of DS. A minority of patients receiving surgery were identified through asymptomatic screening. There was a high complication risk associated with surgery in our study; however, the addition of rigid fixation has lessened the complication rate compared with previous studies. LEVEL OF EVIDENCE: Level IV.

Two novel parameters to evaluate the influence of the age and gender on the anatomic relationship of the atlas and axis in children no more than 8 years old: imaging study.

PURPOSE: Because of the complex cervical vertebral embryology and some normal variations, the atlantoadental interval (ADI) was not suitable for the evaluation of the anatomic relationship between the atlas and axial in children less than 2 years old. And the influence of the age and gender on the anatomic relationship between atlas and axial in children was still unclear. Two novel parameters, atlas-axis anteroposterior distance (AAAD) and atlas-axis lateral distance (AALD), were invented to evaluate the anatomic relationship between the atlas and axis in the children no more than 8 years old with different age and gender. METHODS: Cross-sectional computed tomography (CT) scans of the atlantoaxial joint for 140 randomly selected pediatric patients no more than 8 years old were analyzed. On the ideal CT reconstruction images, AAAD, AALD, atlantoaxial lateral bending angle (AALB), and atlantoaxial rotation angle (AARA) were measured. RESULTS: There was no statistically significant difference between the mean AAAD in different age and gender groups. The 99% confidence interval for AAAD was 7.12-7.82 mm. There was no significant correlation between AAAD and AALB/AARA and AALD and AALB/AARA. CONCLUSION: The AAAD was less than 7.12 mm or much than 7.82 mm that suggested a possible instability in the atlantoaxial joint and could help the diagnosis of the atlantoaxial instability in children no more than 8 years old. There was no difference between the mean AAAD of pediatric patients no more than 8 years old in different age and gender groups.