Anatomical determinants of occipitocervical fusion in skull base chordoma resection: a systematic review of the literature with illustrative cases.

OBJECTIVE: Skull base chordomas are rare, locally osseo-destructive lesions that present unique surgical challenges due to their involvement of critical neurovascular and bony structures at the craniovertebral junction (CVJ). Radical cytoreductive surgery improves survival but also carries significant morbidity, including the potential for occipitocervical (OC) destabilization requiring instrumented fusion. The published experience on OC fusion after CVJ chordoma resection is limited, and the anatomical predictors of OC instability in this context remain unclear. METHODS: PubMed and Embase were systematically searched according to the PRISMA guidelines for studies describing skull base chordoma resection and OC fusion. The search strategy was predefined in the authors' PROSPERO protocol (CRD42024496158). RESULTS: The systematic review identified 11 surgical case series describing 209 skull base chordoma patients and 116 (55.5%) who underwent OC instrumented fusion. Most patients underwent lateral approaches (n = 82) for chordoma resection, followed by midline (n = 48) and combined (n = 6) approaches. OC fusion was most often performed as a second-stage procedure (n = 53), followed by single-stage resection and fusion (n = 38). The degree of occipital condyle resection associated with OC fusion was described in 9 studies: total unilateral condylectomy reliably predicted OC fusion regardless of surgical approach. After lateral transcranial approaches, 4 studies cited at least 50%-70% unilateral condylectomy as necessitating OC fusion. After midline approaches-most frequently the endoscopic endonasal approach (EEA)-at least 75% unilateral condylectomy (or 50% bilateral condylectomy) led to OC fusion. Additionally, resection of the medial atlantoaxial joint elements (the C1 anterior arch and tip of the dens), usually via EEA, reliably necessitated OC fusion. Two illustrative cases are subsequently presented, further exemplifying how the extent of CVJ bony elements removed via EEA to achieve complete chordoma resection predicts the need for OC fusion. CONCLUSIONS: Unilateral total condylectomy, 50% bilateral condylectomy, and resection of the medial atlantoaxial joint elements were the most frequently described independent predictors of OC fusion in skull base chordoma resection. Additionally, consistent with the occipital condyle harboring a significantly thicker joint capsule at its posterolateral aspect, an anterior midline approach seems to tolerate a greater degree of condylar resection (75%) than a lateral transcranial approach (50%-70%) prior to generating OC instability.

[Preoperative Simulation for Complication Control in Spinal Surgery].

The craniovertebral junction not only contains anatomically important structures such as the medulla oblongata, upper cervical spinal cord, and vertebral artery, but also controls the dynamic movements of flexion, extension, and rotation of the head and neck. Consequently, instability and spinal deformities can easily occur in the craniovertebral region, and appropriate treatment should be selected based on the specificity of the lesion. Basilar invagination often involves bone and vascular anomalies and fusion surgery is often required. Therefore, careful pre-operative simulations are necessary. The creation and use of three-dimensional bone models, including image navigation, are useful for surgical simulation.

Anatomy of the craniocervical junction - A review.

An in-depth understanding of the anatomy of the craniocervical junction (CCJ) is indispensable in skull base neurosurgery. In this paper, we discuss the osteology of the occipital bone, the atlas (C1) and axis (C2), the ligaments and the muscle anatomy of the CCJ region and their relationships with the vertebral artery. We will also discuss the trajectory of the vertebral artery and review the anatomy of the jugular foramen and lower cranial nerves (IX to XII). The most important surgical approaches to the CCJ, including the far lateral approach, the anterolateral approach of Bernard George and the endoscopic endonasal approach, will be discussed to review the surgical anatomy.

Novelties for increased safety in cranio-vertebral surgery: a review.

The cranio-vertebral junction (CVJ) was formerly considered a surgical "no man's land" due to its complex anatomical and biomechanical features. Surgical approaches and hardware instrumentation have had to be tailored in order to achieve successful outcomes. Nowadays, thanks to the ongoing development of new technologies and surgical techniques, CVJ surgery has come to be widely performed in many spine centers. Accordingly, there is a drive to explore novel solutions and technological nuances that make CVJ surgery safer, faster, and more precise. Improved outcome in CVJ surgery has been achieved thanks to increased safety allowing for reduction in complication rates. The Authors present the latest technological advancements in CVJ surgery in terms of imaging, biomaterials, navigation, robotics, customized implants, 3D-printed technology, video-assisted approaches and neuromonitoring.

Reduction of Atlanto-Occipital Dislocation After Cervical Collar Removal.

A 39-year-old male pedestrian-hit-by-car was transferred to our institution with atlantooccipital dislocation (AOD) on outside computed tomography (CT) imaging. On arrival he had a rigid cervical collar in place. Given the reported AOD, we placed the patient flat, removed the cervical collar, and supported the head in neutral alignment with sandbags. Due to a technical issue uploading his prior imaging to our system, the patient underwent repeat CTs at our center. Subsequently, after the outside images were uploaded, we were able to compare his cervical spine CT images before and after removing his cervical collar. On comparison, we noted a substantial reduction in AOD after collar removal. We hope this serves as a reminder of this key step in managing a rare but deadly clinical entity and a small demonstration of the efficacy of this intervention.

Radiologic Features of Atlas Occipitalization and Its Clinical Implications.

STUDY DESIGN: Retrospective radiographic analysis. OBJECTIVES: Evaluation of the anatomic features of the craniovertebral junction in patients with occipitalization with and without atlantoaxial dislocation (AAD). SUMMARY OF BACKGROUND DATA: Atlas occipitalization is a common feature of congenital AAD and usually requires surgical intervention. However, not all instances of occipitalization necessarily lead to AAD. No study has specifically examined and compared the craniovertebral bony morphology in occipitalization with, and without, AAD. MATERIALS AND METHOD: We reviewed computed tomography (CT) scans of 2500 adult outpatients. Occipitalization cases without AAD (ON) were selected. Meanwhile, a series of 20 inpatient occipitalization cases with AAD (OD) were obtained in parallel. Another 20 control cases without occipitalization were also included. Multi-directional reconstructed CT images of all cases were analyzed. RESULTS: A total of 18 adults with ON were found in all 2500 outpatients (0.7%). Both anterior height and posterior height of C1 lateral mass in the control group were significantly larger than those in both the ON and OD groups, whereas posterior height in the OD group was significantly less than that in the ON group. Three morphologic types of the occipitalized atlas posterior arch were identified: Type I, bilateral sides were unfused with opisthion; Type II, unilateral side was unfused with opisthion, whereas the other side was fused; and Type III, bilateral sides were fused with opisthion. In the ON group, three cases were type I (17%), six cases were type II (33%), and nine cases were type III (50%). In the OD group, all 20 cases were type III (100%). CONCLUSIONS: Atlas occipitalization with, and without, AAD results from a distinctly different bony morphology at the craniovertebral junction. The novel classification system based on reconstructed CT images may be useful in prognosticating AAD in the setting of atlas occipitalization.

How I do it: lateral approach for craniocervical junction tumors.

BACKGROUND: The far lateral approach has been developed to access lesions at the craniocervical junction and upper cervical spinal canal. Associated morbidity triggered the development of less invasive tailored approaches. METHOD: In this lateral approach to the craniocervical junction, the occipital condyle is kept intact, vertebral artery manipulation is minimized, and the sigmoid sinus is not skeletonized. A linear incision through skin and muscles and use of an abdominal wall fat graft minimize the risk of cerebrospinal fluid leakage. CONCLUSIONS: The exposure provided is sufficient for the majority of tumors in this region and allows for low complication rates.

Factors Affecting the Outcomes of Traumatic Atlanto-Occipital Dislocations in Adults: A Systematic Review.

OBJECTIVE: The objectives of this study were to conduct a systematic review of the literature to determine the optimal treatment method for patients with atlanto-occipital dislocation (AOD) and to identify possible factors influencing their outcomes. METHODS: We conducted a systematic review of the PubMed database between January 1966 and December 2020. The main inclusion criterion was articles that discussed AOD treatment methods, and outcome descriptions were selected for analysis. Intergroup differences were assessed using nonparametric statistical methods. RESULTS: Of the 657 articles identified initially, only 54 met the inclusion criteria, resulting in data from 139 patients. Type I or II AODs were more frequent in patients injured in road traffic accidents, whereas type III AODs were more frequent in patients with catatrauma (P = 0.027). Spinal cord injury was more frequently observed in patients with types I and II AODs than in those with type III AOD (P = 0.026). Improved outcomes were more common in the surgical treatment group (P < 0.001). Significant differences in treatment outcomes between the halo device and orthosis groups were not observed (P = 0.32). CONCLUSIONS: Prognosis of AOD is unfavorable in adults with dislocations resulting from road traffic accidents, those with types I and II AOD, and patients younger than 22 years and older than 47 years. Surgical treatment was optimal for adult patients with an AOD, and treatment outcomes did not depend on the number of occipitocervical fusion levels. Immobilization with the halo device showed no advantages over use of an external orthosis.

Occult craniocervical dissociation on cervical CT: an under-appreciated presentation of craniocervical trauma requiring occipital cervical fusion.

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.

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.

The enigma of acute worsening after a latent interval in post-operative patients of craniovertebral junction instability.

OBJECTIVE: For reasons that are poorly understood and still undocumented, few patients of craniovertebral junction (CVJ) anomalies with instability/atlantoaxial dislocation (AAD) in our practice have been seen to suffer unprecedented acute neurological deterioration with respiratory compromise about 48-72 h post-surgery. This has been specifically observed after an uneventful, stable clinical condition in the immediate postoperative period. This study attempts to elucidate the factors and outcomes related to such latent worsening. PATIENTS AND METHODS: The 4-year surgical data of 268-patients with CVJ instability (AAD/basilar-invagination) who underwent C1-C2 fixation were retrospectively studied. The relevant factors of 17 patients who showed such deterioration were compared with that of the remaining in a multivariate model. RESULTS: Seventeen patients had acute neurological worsening with respiratory arrest after a latent period. Ten improved at follow-up. Contrary to the general expectation, poor preoperative neurological score or severe dislocation that needed considerable manipulation did not influence such worsening. Dural injury in patients with associated Chiari malformation and vertebral arterial (VA) injury emerged as independent factors (p < 0.01) associated with this pattern of delayed neurological decline. CONCLUSIONS: We report our management and outcomes of a unique subset of AAD with neurological worsening in the postoperative period after 48-72 h. Such a subgroup was not previously identified in literature. Dural lacerations in AAD with Chiari, and VA injury seem to predispose to such complications and should be prevented at best. Close observation, and caution regarding early extubation (<72 h) is recommended in this subset despite an apparently uneventful immediate postoperative neurological status.

Posterior percutaneous transarticular stand-alone screw instrumentation of C1-C2 with endoscopic assistance: A report of two cases / Exploración instrumental percutánea posterior con tornillo independiente transarticular en C1-C2 mediante endoscopia: informe de dos casos

We present two cases of minimally invasive posterior transarticular screw fixation of C1-C2. The points for screw insertion were visualized by endoscopy via the instrumental port. A patient with a type III odontoid fracture with subluxation underwent a minimally invasive posterior stand-alone transarticular screw fixation. Despite the application of compression screws, for technical reasons, only minimal compression on the anterior third of the C1-C2 lateral joint was achieved. However, complete fracture fusion was achieved with stable fibrous C1-C2 fusion 2.5 years postoperatively. A second patient with a chronic type II odontoid fracture underwent percutaneous C1-C2 fixation by the same method. After 2 years, fracture fusion and C1-C2 lateral mass ankylosis were achieved. The use of a tubular retractor and endoscopy in stand-alone screw fixation of C1-C2 allows direct visualization of the screw entry point and decreases surgical trauma. This procedure might be an alternative to other methods of transarticular instrumentation (AU)

Presentamos dos casos de fijación posterior artroscópica con tornillo transarticular en C1-C2. Las ubicaciones para la inserción del tornillo se visualizaron mediante un trocar para el endoscopio. Un paciente con una fractura de apófisis odontoides de tipo III con luxación parcial se sometió a fijación posterior artroscópica con tornillo independiente transarticular. A pesar de la aplicación de tornillos de compresión y, por motivos técnicos, solo se logró una compresión mínima del tercio anterior de la articulación lateral C1-C2. Sin embargo, se logró la artrodesis completa de la fractura, con una artrodesis en C1-C2 fibrosa estable después de dos años y medio de la intervención quirúrgica. Un segundo paciente con una fractura de apófisis odontoides de tipo II crónica se sometió a fijación percutánea en C1-C2 con el mismo método. Después de dos años, se logró la artrodesis de la fractura y de la masa lateral en C1-C2. El uso de un separador tubular y de la endoscopia en la fijación con tornillo independiente en C1-C2 permite la visualización directa del punto de entrada del tornillo y disminuye el traumatismo quirúrgico. Este procedimiento puede ser una alternativa a otros métodos de exploración instrumental transarticular (AU)

Anterior Distraction and Reduction with Posterior Stabilization for Basilar Invagination: A Novel Technique.

BACKGROUND: Introduction of a posterior spacer for atlantoaxial joint distraction followed by posterior stabilization is a commonly performed procedure for irreducible atlantoaxial dislocation. We present a unique case in which posterior distraction was associated with increased risk of injury to the vertebral artery (VA) owing to its anomalous course, and hence a novel anterior distraction technique was used. CASE DESCRIPTION: A 45-year-old woman presented with severe neck pain for 1 month with gait imbalance and history of occipital headache for 1 year. Clinical examination revealed upper motor neuron-type findings. Hoffmann sign was positive bilaterally. Clinically, the patient had Nurick grade 4 cervical myelopathy. Magnetic resonance imaging showed basilar invagination along with Arnold-Chiari malformation and syrinx formation at C3-C4 vertebral levels. CT angiography revealed anomalous VAs directly overlying the atlanto-occipital joint. Owing to the anomalous route of the VA and unfavorable slope of facet joints, a 2-step anterior reduction followed by posterior stabilization surgery was planned. We achieved complete reduction using a 10-mm titanium cage inserted via a retropharyngeal approach. Following anterior reduction, instrumented in situ occipitocervical fusion was performed using a plate and screw construct. At 2-year follow-up, the patient is ambulating independently without gait imbalance and with successful radiologic fusion. CONCLUSIONS: The craniovertebral junction has a unique pathoanatomy, and the course of the vertebral artery is variable. Appropriate investigations, including computed tomography angiography, with adequate surgical planning will provide a desirable long-term outcome. Our novel technique has the potential to add a new dimension to the management of irreducible atlantoaxial dislocation.

Traumatic atlanto-occipital dislocation in children: is external immobilization an option?

OBJECT: Traumatic atlanto-occipital dislocation (AOD) is a relatively uncommon traumatic cervical spine injury characterized by disruption and instability of the atlanto-occipital joint. At many centers, management of pediatric AOD includes occipitocervical arthrodesis, but whether external immobilization without surgery is a viable treatment option for some pediatric patients is unknown. To answer this question, we analyzed our outcomes of pediatric AOD at the Hospital for Sick Children. METHODS: We performed a retrospective chart review of all children with clinical and radiographic evidence of traumatic AOD. A total of 10 patients met criteria for traumatic AOD: 8 were treated with external immobilization alone and 2 were treated with occipitocervical arthrodesis. RESULTS: Eight patients were treated exclusively with 3 months of halo immobilization. Two patients were treated with occipitocervical instrumentation and arthrodesis. No patient undergoing halo immobilization required subsequent operative fusion. CONCLUSION: Halo immobilization is a safe, viable, and definitive treatment option for the selected children with AOD.

Management of post-traumatic craniovertebral junction dislocation: A PRISMA-compliant systematic review and meta-analysis of casereports.

Although historically considered fatal, with the advent of improved pre-hospital care, traumatic dislocation of the craniovertebral junction (CVJ) has been increasing in prevalence in neurosurgical centers. As more survivors are reported each year, a timely review with meta-analysis of their management seems necessary. PRISMA checklist was followed step by step. PubMed and Web of Science databases were searched using words "craniovertebral junction dislocation" and their corresponding synonyms. Study eligibility criteria included research studies from 2015 onwards that delineated adult and pediatric patients with confirmed post-traumatic atlantooccipital dislocation (AOD) or atlantoaxial dislocation (AAD) who survived until proper treatment. Of 1475 initial records, 46 articles met eligibility criteria with a total of 141 patients with traumatic CVJ dislocation. Of the patients, 90 were male (63.8%). Mean age of the cohort was 33.3 years (range 1-99 years). Trauma that most often led to this injury was road traffic accident (70.9%) followed by falls (24.6%). The majority of authors support posterior instrumentation of C1-C2 (45.2%) especially by means of Goel-Harms method. At mean follow-up of 15.4 months (range 0.5-60 months), 27.2% of treated patients remained neurologically intact. Of initially symptomatic, 59% improved, 37% were stable, and 4% deteriorated. Instrumenting the occiput in cases of pure AAD was associated with lower chance of neurological improvement in chi-square test (p = 0.0013) as well as in multiple linear regression (ß = - 0.3; p = 0.023). The Goel-Harms C1-C2 fusion is currently the most frequently employed treatment. Many survivors remain with no deficits or improve, rarely deteriorate. Involving the occiput in stabilization in cases of AAD without AOD might be related with worse neurological prognosis.

Two-Stage Approach for Unstable Pediatric Craniocervical Junction Anomalies with a Halo Vest and Delayed Occipitocervical Fusion: Technical Note, Case Series, and Literature Review.

OBJECTIVE: Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes. METHODS: A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis. RESULTS: Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from -3.26 mm to -6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41). CONCLUSIONS: The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

A 360° Approach to the Craniovertebral Junction in a Cadaveric Laboratory Setting: Historical Insights, Current, and Future Perspectives in a Comparative Study.

BACKGROUND: We herein outline the experience matured in our equipped Cranio-Vertebral Junction Laboratory for anatomic dissection. METHODS: An extreme lateral approach (ELA) was performed on 4 fresh cadavers and submandibular approach was performed on 5. An endoscope and navigation-assisted far lateral approach (FLA) was performed in 5 injected specimens. In these specimens, a transoral approach was also performed, as well as a neuronavigation-assisted comparison between transoral and transnasal explorable distances. RESULTS: As calculated with neuronavigation, statistically significant differences both in the explored craniocaudal (P = 0.003) and lateral (P = 0.008) distances were observed between the transoral approach and endoscopic endonasal approach. In FLA, neuronavigation facilitated identification and partial removal of the occipital condyle; in one case, during endoscopic intradural exploration, tearing of the emerging roots of the 11th cranial nerve occurred. In ELA, the site where the accessory nerve pierces into the sternocleidomastoid muscle was found at a distance from the tip of the mastoid between 3 and 4 cm. CONCLUSIONS: During dissections, as in the clinical setting, endoscope and image guidance give the surgeon a constant orientation, increasing the accuracy and the safety of the approach. Nonetheless, the encumbrance of the endoscope could represent a limit in deep and narrow corridors as those running across the craniovertebral junction, especially in "oblique" FLA and ELA, in which the surgical target is often hidden by a delicate tangle of nerves and vessels. Its use appears more suitable and safer in "straight" approaches as transoral and transnasal in which there are no neurovascular structures interposed.

Atlanto-occipital Dissociation in the Setting of Relatively Normal Radiologic Findings.

BACKGROUND: Craniocervical junction (CCJ) dislocations are often fatal. Atlanto-occipital dissociation can be challenging to diagnose, especially in patients who present with absent or subtle radiologic signs. CASE DESCRIPTION: A neurologically intact 37-year-old patient presented to the hospital following a high-speed motor vehicle accident. Initial computed tomography scans showed normal CCJ anatomy, but magnetic resonance imaging (MRI) of the CCJ was performed to further evaluate perimesencephalic subarachnoid hemorrhage. MRI revealed partial disruption of the anterior atlantoaxial membrane and tectorial membrane as well as complete disruption of the posterior atlanto-occipital membrane, ligamentum flavum, and apical ligament, signifying atlanto-occipital dissociation. Halo spinal immobilization was performed in preparation for stabilization with posterior occipitocervical fusion; however, the CCJ distracted widely during surgery owing to the accident-related dislocation, signifying an unstable fracture. Posterolateral fusion was performed, and the distraction injury was corrected via posterior surgical instrumentation. CONCLUSIONS: Normal occiput-C1 craniometric parameters in the setting of unexplained perimesencephalic subarachnoid hemorrhage does not eliminate the possibility of missed or delayed diagnosis of traumatic atlanto-occipital dissociation injuries. Cervical MRI without contrast should be considered in patients with vertebral artery dissection or perimesencephalic subarachnoid hemorrhage after a blunt injury with neck pain. When MRI shows evidence of disruption of ≥2 atlanto-occipital ligaments, surgical stabilization should be considered, as these are clinically very unstable injuries.

Congenital Fusion of Dens to T3 Vertebra in Klippel-Feil Syndrome.

BACKGROUND: Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion. CASE DESCRIPTION: Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement. CONCLUSION: This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.