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.

Dysphagia, health-related quality of life, and return to work after occipitocervical fixation.

PURPOSE: The purpose of this study was to evaluate patient-reported outcome measures (PROMS) on dysphagia, health-related quality of life (HRQoL) and return to work after occipitocervical fixation (OCF). Postoperative radiographic measurements were evaluated to identify possible predictors of dysphagia. METHODS: All individuals (≥ 18 years) who underwent an OCF at the study center or were registered in the Swedish spine registry (Swespine) between 2005 and 2019, and were still alive when the study was conducted, were eligible for inclusion. There was no overlap between the cohorts. Prospectively collected data on dysphagia (Dysphagia Short Questionnaire DSQ), HRQoL (EQ5D-3L) and return to work were used. Radiological and baseline patient data were retrospectively collected. In addition, HRQoL data of a matched sample of individuals was elicited from the Stockholm Public Health Survey 2006. RESULTS: In total, 54 individuals were included. At long-term follow-up, 26 individuals (51%) had no dysphagia, and 25 (49%) reported some degree of dysphagia: 11 (22%) had mild dysphagia, and 14 (27%) had moderate to severe dysphagia. On a group level, the OCF sample scored significantly lower EQVAS and EQ-5Dindex values compared to the general population (60.0 vs. 80.0, p = 0.016; 0.43 vs. 0.80, p < 0.001). Individuals working preoperatively returned to work after surgery. Of those responding, 88% stated that they would undergo the OCF operation if it was offered today. No predictors of dysphagia based on radiographic measurements were identified. CONCLUSION: Occipitocervical fixation results in a high frequency of long-term dysphagia. The HRQoL of OCF patients is significantly reduced compared to matched controls. However, most patients are satisfied with their surgery. No radiographic predictors of long-term dysphagia could be identified. Future prospective and systematic studies with larger samples and more objective outcome measures are needed to elucidate the causes of dysphagia in OCF.

MR documented craniocervical ligamentous injury at age 18 months: delayed formation of OS odontoideum. Complex management issues. Case-based review.

OBJECTIVE: There are two separate theories regarding the genesis of os odontoideum: congenital and post-traumatic. Trauma documentation in the past has been the presence of a normal odontoid process at the time of initial childhood injury and subsequent development of the os odontoideum. True MR documentation of craniocervical injury in early childhood and subsequent os odontoideum formation has been very rare. METHODS: An 18-month-old sustained craniocervical ligamentous injury documented on MRI with transient neurological deficit. Chiari I abnormality was also recorded. Subsequent serial imaging of craniocervical region showed the formation of os odontoideum and instability. He became symptomatic from the os odontoideum and the Chiari I abnormality. The patient underwent decompression and intradural procedure for Chiari I abnormality and occipitocervical fusion. Postoperative course was complicated by the failure of fusion and redo. He later required transoral ventral medullary decompression. He recovered. RESULTS: This is an MR documented craniocervical ligamentous injury with sequential formation of os odontoideum with accompanying changes in the atlas. Despite a subsequent successful dorsal occipitocervical fusion, he became symptomatic requiring transoral decompression. CONCLUSIONS: Os odontoideum here is recognized as a traumatic origin with the presence of congenital Chiari I abnormality as a separate entity. The changes of the anterior arch of C1 as well as the os formation were serially documented and give credence to blood supply changes in the os and atlas as a result of the trauma. The recognized treatment of dorsal occipitocervical fusion failed in this case requiring also a ventral decompression of the medulla.

Anterior occipital condyle screw placement through the endonasal corridor: proof of concept study with cadaveric analysis.

PURPOSE: Odontoidectomy for ventral compressive pathology may result in O-C1 and/or C1-2 instability. Same-stage endonasal C1-2 spinal fusion has been advocated to eliminate risks associated with separate-stage posterior approaches. While endonasal methods for C1 instrumentation and C1-2 trans-articular stabilization exist, no hypothetical construct for endonasal occipital instrumentation has been validated. We provide an anatomic description of anterior occipital condyle (AOC) screw endonasal placement as proof-of-concept for endonasal craniocervical stabilization. METHODS: Eight adult, injected cadaveric heads were studied for placing 16 AOC screws endonasally. Thin-cut CT was used for registration. After turning a standard inferior U-shaped nasopharyngeal flap endonasally, 4 mm × 22 mm AOC screws were placed with a 0° driver using neuronavigation. Post-placement CT scans were obtained to determine: site-of-entry, measured from the endonasal projection of the medial O-C1 joint; screw angulation in sagittal and axial planes, proximity to critical structures. RESULTS: Average site-of-entry was 6.88 mm lateral and 9.74 mm rostral to the medial O-C1 joint. Average angulation in the sagittal plane was 0.16° inferior to the palatal line. Average angulation in the axial plane was 23.97° lateral to midline. Average minimum screw distances from the jugular bulb and hypoglossal canal were 4.80 mm and 1.55 mm. CONCLUSION: Endonasal placement of AOC screws is feasible using a 0° driver. Our measurements provide useful parameters to guide optimal placement. Given proximity of hypoglossal canal and jugular bulb, neuronavigation is recommended. Biomechanical studies will ultimately be necessary to evaluate the strength of AOC screws with plate-screw constructs utilizing endonasal C1 lateral mass or C1-2 trans-articular screws as inferior fixation points.

Surgical treatment of craniocervical instability: comparison of two constructs regarding clinical and radiological outcomes of 100 patients.

PURPOSE: There is an increased demand for surgical solutions to treat craniocervical instability. This retrospective study demonstrates the clinical and radiological outcomes of unstable craniocervical junction treated with occipitocervical fusion. MATERIAL AND METHODS: The mean age of 52 females and 48 males was 56.89 years. The clinical and radiological outcomes were assessed, including NDI, VAS, ASIA score, imaging, complications and bony fusion in two used constructs: a modern occipital plate-rod-screw system (n = 59) and previous bilateral contoured titanium reconstruction plates-screws (n = 41). RESULTS: Clinically and on imaging, patients presented with neck pain, myelopathy, radiculopathy, vascular symptoms and craniocervical instability. The mean follow-up was 6.47 years. A solid bony fusion was achieved in 93.81% of the patients. The NDI and the VAS improved significantly from 28.3 and 7.67 at the presentation to 16.2 and 3.47 at the final follow-up. The anterior and posterior atlantodental interval (AADI and PADI), the clivus canal angle (CCA), the occipitoaxial angle (OC2A) and the posterior occipitocervical angle (POCA) improved significantly. Six patients required early revision. CONCLUSION: Occipitocervical fusion can yield excellent results regarding clinical improvement and long-term stability with a high fusion rate. Simple reconstruction plates, though more demanding surgically, achieve similar results. Preserving a neutral patient's position for fixation avoids postoperative dysphagia and may help prevent adjacent segment disease development.

Radiographic measurements for the prediction of dysphagia after occipitocervical fusion: a systematic review.

BACKGROUND: Occipitocervical fusion (OCF) is a procedure performed for multiple upper cervical pathologies. A common postprocedural complication of OCF is dysphagia, which has been linked to the narrowing of the pharyngeal space due to fixation in a hyper-flexed angle. Postoperative dysphagia is linked to reduced quality of life, prolonged hospital stay, aspiration pneumonia, and increased mortality. This has led to investigations of the association between sagittal radiographic angles and dysphagia following OCF. METHODS: A systematic review of the literature was performed to explore the current evidence regarding cervical sagittal radiographic measurements and dysphagia following OCF. A search strategy was carried out using the PubMed, Embase, and Web of Science databases from their dates of inception until August 2022. Only original English-language studies were considered. Moreover, studies had to include the correlation between dysphagia and at least one radiographic measurement in the sagittal plane. RESULTS: The search and subsequent selection process yielded eight studies that were included in the final review, totaling 329 patients in whom dysphagia had been assessed and graded. The dysphagia score by Bazaz et al. (Spine 27, 22:2453-2458, 2002) was used most often. The pooled incidence of dysphagia, in the early postoperative period, was estimated at 26.4%. At long-term follow-up (range: 17-72 months), about one-third of patients experienced resolution of symptoms, which resulted in a long-term post-OCF dysphagia incidence of 16.5%. Across the studies included, six different radiographic parameters were used to derive several measures which were repeatedly and significantly associated with the occurrence of dysphagia. CONCLUSIONS: The high incidence of postoperative dysphagia following OCF warrants close monitoring of patients, especially in the short-term postoperative period. These patients may be assessed through standardized tools where the one by Bazaz et al. was the most commonly used. Moreover, there are several radiographic measurements that can be used to predict the occurrence of dysphagia. These findings may serve as a basis for strategies to prevent the occurrence of dysphagia after OCF.

Posterior two-step distraction and reduction for basilar invagination with atlantoaxial dislocation: a novel technique for precise control of reduction degree without traction.

PURPOSE: The pathological changes of basilar invagination (BI) and atlantoaxial dislocation (AAD) include vertical and horizontal dislocations. Current surgical techniques have difficulty in accurately controlling the degree of reduction in these two directions and often require preoperative traction, which increases patients' pain, hospital stay, and medical cost. This study aimed to introduce a novel technique for accurately reducing horizontal and vertical dislocation without preoperative traction and report the radiological and clinical outcomes. METHODS: From 2010 to 2020, patients with BI and AAD underwent posterior two-step distraction and reduction (TSDR) and occipitocervical fixation. Radiological examination was used to evaluate the reduction degree (RD) and compression. Japanese Orthopedic Association (JOA) score was used to evaluate clinical outcome. RESULTS: A total of 55 patients with BI and AAD underwent TSDR and occipitocervical fusion. The clinical symptoms of 98.2% of them improved. JOA score increased significantly after the operation. Appropriate (50% ≤ RD < 80%) or satisfactory (RD ≥ 80%) horizontal reduction was achieved in 92.7% of patients, and 90.9% obtained appropriate or satisfactory vertical reduction. Thirty-one patients did not undergo preoperative skull traction. There was no significant difference in radiological outcomes or JOA scores between the traction and non-traction groups. However, the length of hospital stay in the traction group was longer than that in the non-traction group. CONCLUSION: TSDR enables horizontal and vertical reduction. It is a safe, simple, and effective technique for patients with BI and AAD. Despite the absence of preoperative skull traction, the degree of reduction and clinical outcomes were satisfactory.

The predictive ability of occipital to C3 angle for dysphagia after occipitocervical fusion in patients with combined C2-3 Klippel-Feil syndrome.

BACKGROUND: Improper occipitocervical alignment after occipitocervical fusion (OCF) may lead to devastating complications, such as dysphagia and/or dyspnea. The occipital to C2 angle (O-C2a), occipital and external acoustic meatus to axis angle (O-EAa) have been used to evaluate occipitospinal alignment. However, it may be difficult to identify the inferior endplate of the C2 vertebra in patients with C2-3 Klippel-Feil syndrome (KFS). The purpose of this study aimed to compare four different parameters for predicting dysphagia after OCF in patients with C2-3 KFS. METHODS: There were 40 patients with C2-3 KFS undergoing OCF between 2010 and 2019. Radiographs of these patients were collected to measure the occipital to C3 angle (O-C3a), O-C2a, occipito-odontoid angle (O-Da), occipital to axial angle (Oc-Axa), and narrowest oropharyngeal airway space (nPAS). The presence of dysphagia was defined as the patient complaining of difficulty or excess endeavor to swallow. Patients were divided into two groups according to whether they had postoperative dysphagia. We evaluated the relationship between each of the angle parameters and nPAS and analyzed their influence to the postoperative dysphagia. RESULTS: The incidence of dysphagia after OCF was 25% in patients with C2-3 KFS. The Oc-Axa, and nPAS were smaller in the dysphagia group compared to non-dysphagia group at the final follow-up (p < 0.05). Receiver-operating characteristic (ROC) curves showed that dO-C3a had the highest accuracy as a predictor of the dysphagia with an area under the curve (AUC) of 0.868. The differences in O-C3a, O-C2a, O-Da, and Oc-Axa were all linearly correlated with nPAS scores preoperatively and at the final follow-up within C2-3 KFS patients, while there was a higher R2 value between the dO-C3a and dnPAS. Multiple linear regression analysis showed that the difference of O-C3a was the only significant predictor for dnPAS (ß = 0.670, p < 0.001). CONCLUSIONS: The change of O-C3a (dO-C3a) is the most reliable indicator for evaluating occipitocervical alignment and predicting postoperative dysphagia in C2-3 KFS patients. Moreover, dO-C3a should be more than - 2° during OCF to reduce the occurrence of postoperative dysphagia.

Safety analysis and complications of condylar screws in a single-surgeon series of 250 occipitocervical fusions.

OBJECTIVE: Condylar screw fixation is a rescue technique and an alternative to the conventional configuration of occipitocervical fusion. Condylar screws are utilized when previous surgical bone removal along the supraocciput has occurred which makes anchoring of a traditional barplate technically difficult or impossible. However, the challenging dissection of C0-1 necessary for condylar screw fixation and the concerns about possible complications have, thus far, prevented the acquisition of large surgical series utilizing occipital condylar screws. In the largest case series to date, this paper aims to evaluate the safety profile and complications of condylar screw fixation for occipitocervical fusion. METHODS: A retrospective safety and complication-based analysis of occipitocervical fusion via condylar screws fixation was performed. RESULTS: A total of 250 patients underwent occipitocervical fusions using 500 condylar screws between September 2012 and September 2018. No condylar screw pullouts, or vertebral artery impingements were observed in this series. The sacrifice of condylar veins during the dissection at C0-1 did not cause any venous stroke. Hypotrophic condyles were found in 36.4% (91 of the 250) cases and did not prevent the insertion of condylar screws. Two transient hypoglossal deficits occurred at the beginning of this surgical series and were followed by recovery a few months later. Corrective strategies were effective in preventing further hypoglossal injuries. CONCLUSIONS: This surgical series suggests that the use of condylar screws fixation is a relatively safe and reliable option for OC fusion in both adult and pediatric patients. Methodical dissection of anatomical landmarks, intraoperative imaging, and neurophysiologic monitoring allowed the safe execution of the largest series of condylar screws reported to date. Separate contributions will follow in the future to provide details about the long-term clinical outcome of this series.

Spontaneous morphological remodelling of the O-C1 joint after posterior fusion for occipitocervical dislocation.

BACKGROUND: Traumatic occipitocervical dislocation (OCD) occurs due to fatal high-energy injury. Modern screw-based constructs enable successful reduction and stabilisation. In view of this, there are no previous reports on the spontaneous remodelling of the O-C1 joint after posterior fusion. We report the first case of postoperative spontaneous remodelling and stabilisation of the O-C1 joint after traumatic OCD.Case description: A 9-year-old girl suffered from traumatic OCD, accompanied by complete rupture of the O-C1-C2 ligamentous complex. Halo-vest fixation, and subsequently posterior fusion surgery from the occipital bone to C2, with autologous iliac crest bone graft and an allograft were performed. However, we could not achieve complete reduction of the O-C1 joint during surgery owing to extremely severe instability.Postoperative X-ray and computed tomography scan showed incomplete reduction of the O-C1 joint. Insufficient congruity of the O-C1 joint persisted. Afterwards, gradual spontaneous remodelling of the O-C1 joint occurred, both anteriorly and posteriorly 3 months postoperatively. Solid union was achieved 6 months postoperatively. Two years later, bilateral O-C1 joints in the patient were completely reformed and restabilised by incredible vigorous remodelling. Insufficient reduction and persisting poor joint congruence after surgery for OCD was probably restabilised by further spontaneous remodelling of articular morphology in such a young patient. CONCLUSIONS: Postoperative spontaneous remodelling of the O-C1 joint after posterior reconstruction for OCD may occur in young patients. Incomplete reduction of the O-C1 joint during surgery may be acceptable due to the possibility of postoperative bone remodelling and restabilisation.

A novel COL2A1 mutation causing spondyloepiphyseal dysplasia congenita in a Chinese family.

BACKGROUND: Spondyloepiphyseal dysplasia congenita is an autosomal dominant cartilaginous dysplasia characterized by short trunk, abnormal epiphysis, and flattened vertebral body. Skeletal features of SEDC are present at birth and evolve over time. Other features of SEDC include myopia and/or retinal degeneration with retinal detachment and cleft palate. A mutation in the COL2A1 gene located in 12q13.11 is considered as one of the important causes of SEDC. In 2016, Barat-Houari et al. reported a large number of COL2A1 mutations. Among them, a non-synonymous mutation in COL2A1 exon 37, c.2437G>A (p. Gly813Arg), has been reported to cause SEDC in only one patient from France so far. METHODS: We followed up a patient with SEDC phenotype and his family members. The clinical manifestations, physical examination and imaging examination, including X-ray, CT and MRI, were recorded. The whole-exome sequencing was used to detect the patients' genes, and the pathogenic genes were screened out by comparing with many databases. RESULTS: We report a Chinese patient with SEDC phenotype characterized by short trunk, abnormal epiphysis, flattened vertebral body, narrow intervertebral space, dysplasia of the odontoid process, chicken chest, scoliosis, hip and knee dysplasia, and joint hypertrophy. Gene sequencing analysis showed that the patient had a heterozygous mutation (c.2437G>A; p. Gly813Arg) in the COL2A1 gene. No COL2A1 mutation or SEDC phenotype was observed in his family members. This is the first report of SEDC caused by this mutation in an East Asian family. CONCLUSION: This report provides typical clinical, imaging, and genetic evidence for SEDC, confirming that a de novo mutation in the COL2A1 gene, c.2437G>A (p. Gly813Arg), causes SEDC in Chinese population.

Posterolateral epidural supra-C2-root approach (PESCA) for biopsy of lesions of the odontoid process in same sitting after occipitocervical fixation and decompression-perioperative management and how to avoid vertebral artery injury.

This study aims to describe the posterolateral epidural supra-C2-root approach (PESCA), which might be a good alternative to the transoral, anterolateral, and other posterolateral approaches for biopsy of lesions of the odontoid process (OP). The preoperative planning of PESCA included computerized tomography (CT), CT-angiography, and three-dimensional reconstruction (if possible, even with three-dimensional print) to analyze the angle of the trajectory and the anatomy of the vertebral artery (VA). For PESCA, the patient is positioned under general anesthesia in prone position. In case of an osteolytic lesion with fracture of the OP, an X-ray is performed after positioning to verify anatomic alignment. In the first step, in case of instability and compression of the spinal cord, a craniocervical fusion and decompression is performed (laminectomy of the middle part of the C1 arc and removal of the lower part of the lateral C1 arc). The trajectory is immediately above the C2 root (and under the upper rest of the lateral part of C1 arc). Even if the trajectory is narrowed, it is possible to perform PESCA without relevant traction of the spinal cord. The vertical segment of V3 of the VA at the level of C2 is protected by the vertebral foramen, and the horizontal part of V3 is protected by the remnant upper lateral part of the C1 arc (in case of normal variants). PESCA might be a good choice for biopsy of selected lesions of the OP in same sitting procedure after craniocervical stabilization and decompression.

Predictive ability of pharyngeal inlet angle for the occurrence of postoperative dysphagia after occipitocervical fusion.

BACKGROUND: PIA has been proven to be a predictor for postoperative dysphagia in patients who undergo occipitospinal fusion. However, its predictive effect for postoperative dysphagia in patients who undergo OCF is unknown. The aim of this study was to evaluate the predictive ability of the pharyngeal inlet angle (PIA) for the occurrence of postoperative dysphagia in patients who undergo occipitocervical fusion (OCF). METHODS: Between 2010 and 2018, 98 patients who had undergone OCF were enrolled and reviewed. Patients were divided into two groups according to the presence of postoperative dysphagia. Radiographic parameters, including the atlas-dens interval (ADI), O-C2 angle (O-C2a), occipital and external acoustic meatus to axis angle (O-EAa), C2 tilting angle (C2Ta), C2-7 angle (C2-7a), PIA and narrowest oropharyngeal airway space (nPAS), were measured and compared. Simple linear regression and multiple regression analysis were used to evaluate the radiographic predictors for dysphagia. In addition, we used PIA = 90° as a threshold to analyze its effect on predicting dysphagia. RESULTS: Of the 98 patients, 26 exhibited postoperative dysphagia. Preoperatively, PIA in the dysphagia group was significantly higher than that in the nondysphagia group. We detected that O-C2a, O-EAa, PIA and nPAS all decreased sharply in the dysphagia group but increased slightly in the nondysphagia group. The changes were all significant. Through regression analyses, we found that PIA had a similar predictive effect as O-EAa for postoperative dysphagia and changes in nPAS. Additionally, patients with an increasing PIA exhibited no dysphagia, and the sensitivity of PIA <90° in predicting dysphagia reached 88.5%. CONCLUSIONS: PIA could be used as a predictor for postoperative dysphagia in patients undergoing OCF. Adjusting a PIA level higher than the preoperative PIA level could avoid dysphagia. For those who inevitably had decreasing PIA, preserving intraoperative PIA over 90° would help avert postoperative dysphagia. TRIAL REGISTRATION: This trial has been registered in the Medical Ethics Committee of West China Hospital, Sichuan University. The registration number is 762 and the date of registration is Sep. 9 th, 2019.

Feasibility of occipital condyle screw placement in patients with Chiari malformation type I: a computed tomography-based morphometric study.

BACKGROUND: The occipital condyle (OC) screw is an alternative technique for occipitocervical fixation that is especially suitable for revision surgery in patients with Chiari malformation type I (CMI). This study aimed to investigate the feasibility and safety of this technique in patients with CMI. METHODS: The CT data of 73 CMI patients and 73 healthy controls were retrospectively analyzed. The dimensions of OCs, including length, width, height, sagittal angle, and screw length, were measured in the axial, sagittal, and coronal planes using CT images. The OC available height was measured in the reconstructed oblique parasagittal plane of the trajectory. RESULTS: The mean length, width, and height of OCs in CMI patients were 17.79 ± 2.31 mm, 11.20 ± 1.28 mm, and 5.87 ± 1.29 mm, respectively. All OC dimensions were significantly smaller in CMI patients compared with healthy controls. The mean screw length and sagittal angle were 19.13 ± 1.97 mm and 33.94° ± 5.43°, respectively. The mean OC available height was 6.36 ± 1.59 mm. According to criteria based on OC available height and width, 52.1% (76/146) of OCs in CMI patients could safely accommodate a 3.5-mm-diameter screw. CONCLUSIONS: The OC screw is feasible in approximately half of OCs in CMI patients. Careful morphometric analyses and personalized surgical plans are necessary for the success of this operation in CMI patients.

Occipitocervical fusion complicated with cerebellar abscess: a case report.

BACKGROUND: Occipitocervical (OC) fusion is indicated for OC instability and other conditions. Surgical complications include infection, malunion, and instrument failure. CASE PRESENTATION: We described a patient who underwent OC fusion and subsequently developed complication of cerebellar abscess and obstructive hydrocephalus. A 63-year-old male patient had been suffering from long-term neck pain and limb numbness and weakness. Cervical spine examination revealed tight stenosis at C1 level and instability in the C1-C2 joints. A C1 laminectomy with OC fusion was performed, and the patient was discharged. Unfortunately, a few days later, he went to the emergency department and complained of persistent dizziness, vomiting, and unsteady gait. Computed tomography (CT) and magnetic resonance imaging (MRI) images revealed a suspicious cerebellar abscess formation and hydrocephalus. Furthermore, CT images indicated that the left screw was loose, and the diameter of the right screw hole was much larger than the size of the screw. Besides, inappropriate length of the screw penetrated the occipital bone and may cause the disruption of dura mater. The patient underwent external ventricular drainage first, followed by abscess drainage and C1-C2 fixation a few days later. He was discharged without any further neurological deficits or infectious problems. The patient recovered with intact consciousness, full muscle strength, and improved numbness throughout the extremities, with a Nurick grade of 1. A follow-up magnetic resonance imaging at 3 months after surgery revealed near total resolution of the abscess. Inform consent was obtained from this patient. CONCLUSIONS: Carefully conducting the procedure using the most tailored approach is essential to successful surgery, but this rare complication should always be kept in mind.

Surgical treatment for basilar invagination with irreducible atlantoaxial dislocation: transoral atlantoaxial reduction plate fixation vs occipitocervical fixation.

BACKGROUND: Transoral atlantoaxial reduction plate (TARP) fixation or occipitocervical fixation (OF) is an effective treatment for basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD). But, all current clinical studies involved a single surgical procedure. The clinical effects of TARP and OF operation for BI with IAAD have yet to be compared. We therefore present this report to compare the treatment of TARP and OF procedure for BI with IAAD. METHODS: Fifty-six patients with BI with IAAD who underwent TARP or OF operation from June 2011 to June 2017 were retrospectively analyzed. Among these, 35 patients underwent TARP operation (TARP group), and 21 patients underwent OF operation (OF group). We compared the difference of clinical, radiological, and surgical outcomes between the TARP and OF groups postoperatively. RESULTS: Compared with OF group, the operative time and blood loss in TARP group were lower. There was no statistical difference in the atlantodental interval (ADI), clivus canal angle (CCA), cervicomedullary angle (CMA), distance between the top of the odontoid process and the Chamberlain line (CL) and Japanese Orthopaedic Association (JOA) score between the TARP and OF groups preoperatively, but the improvements of these parameters in the TARP group were superior to those in the OF group postoperatively. The fusion rates were higher in the TARP group than those in the OF group at the early stage postoperatively. CONCLUSIONS: TARP and OF operations are effective surgical treatment for BI with IAAD, but the performance of reduction and decompression and earlier bone fusion rates of TARP procedure are superior to those of OF.

Atlanto-occipital dissociation.

INTRODUCTION: Atlanto-occipital dissociation (AOD) is a rare and unstable injury of the craniocervical junction, associated with very high morbidity and mortality. The most common cause of this injury is high energy trauma with hyperextension of the cranium, such as car accidents. Due to specific anatomical predispositions, children and young adults are the frequently affected populations. Improving pre-hospital and early emergency care has resulted in a higher sensitivity of AOD diagnosis. METHODS: A retrospective analysis of all patients with cervical spine trauma, treated at the Masaryk Hospital Trauma Center between 2008 and 2018, identified 7 patients with AOD. The cohort consisted of 5 males and 2 females, with a mean age of 19,6 years and with the age range 9 to 35 years. All cases occurred as a result of a car accident. RESULTS: All patients in the cohort had findings of a highly unstable C0-C1 injury on their CT scans on admission. Four patients died early, while undergoing CPR in the emergency department. Two patients were in severe neurological states, with lesions of the upper cervical spinal cord and medulla oblongata on MRI. These patients were treated with external halo fixation and died within 3 days of the trauma. Only one patient with a new progressive neurological deficit was successfully treated using acute occipitocervical stabilization and fusion. CONCLUSION: The increasing incidence of AOD requires an early diagnosis, which minimizes the risk of successive clinical deterioration. The diagnostic method of choice is the C1-condyle interval (CCI) CT assessment along with cervical spine MRI. Standard treatment of stable patients with unstable AOD injuries consists in posterior occipitocervical stabilization and fusion of C0-C2.

Instrumented arthrodesis for non-traumatic craniocervical instability in very young children.

PURPOSE: Occipitocervical instrumentation is infrequently required for stabilization of the axial and subaxial cervical spine in very young children. However, when it is necessary, unique surgical considerations arise in children when compared with similar procedures in adults. METHODS: The authors reviewed literature describing fusion of the occipitocervical junction (OCJ) in toddlers and share their experience with eight cases of young children (age less than or equal to 4 years) receiving occiput to axial or subaxial spine instrumentation and fixation. Diagnoses and indications included severe or secondary Chiari malformation, skeletal dysplastic syndromes, Klippel-Feil syndrome, Pierre Robin syndrome, Gordon syndrome, hemivertebra and atlantal occipitalization, basilar impression, and iatrogenic causes. RESULTS: All patients underwent occipital bone to cervical spine instrumentation and fixation at different levels. Constructs extended from the occiput to C2 and T1 utilizing various permutations of titanium rods, autologous rib autografts, Mersilene sutures, and combinations of autografts with bone matrix materials. All patients were placed in rigid cervical bracing or halo fixation postoperatively. No postoperative neurological deficits or intraoperative vascular injuries occurred. CONCLUSION: Instrumented arthrodesis can be a treatment option in very young children to address the non-traumatic craniocervical instability while reducing the need for prolonged external halo vest immobilization. Factors affecting fusion are addressed with respect to preoperative, intraoperative, and postoperative decision-making that may be unique to the toddler population.

Occipitocervical Fusion: An Updated Review.

Occipitocervical fusion (OCF) is indicated for instability at the craniocervical junction (CCJ). Numerous surgical techniques, which evolved over 90 years, as well as unique anatomic and kinematic relationships of this region present a challenge to the neurosurgeon. The current standard involves internal rigid fixation by polyaxial screws in cervical spine, contoured rods and occipital plate. Such approach precludes the need of postoperative external stabilization, lesser number of involved spinal segments, and provides 95-100% fusion rates. New surgical techniques such as occipital condyle screw or transarticular occipito-condylar screws address limitations of occipital fixation such as variable lateral occipital bone thickness and dural sinus anatomy. As the C0-C1-C2 complex is the most mobile portion of the cervical spine (40% of flexion-extension, 60% of rotation and 10% of lateral bending) stabilization leads to substantial reduction of neck movements. Preoperative assessment of vertebral artery anatomical variations and feasibility of screw insertion as well as visualization with intraoperative fluoroscopy are necessary. Placement of structural and supplemental bone graft around the decorticated bony elements is an essential step of every OCF procedure as the ultimate goal of stabilization with implants is to provide immobilization until bony fusion can develop.

Post-operative regression of retro-odontoid pseudotumors treated with and without fusion.

PURPOSE: Retro-odontoid pseudotumor is common in elderly people and is a cause of cervical myelopathy. The goal of the study was to investigate surgical procedures, outcomes, and post-operative spontaneous regression of posterior cervical retro-odontoid pseudotumors. METHODS: The subjects were 29 patients who underwent surgery for myelopathy due to a retro-odontoid pseudotumor around the craniocervical region at 9 facilities and were followed-up for an average of 54 months (range 12-96 months). Data were collected in a multicenter review of a retrospective database. Comparisons were performed between cases treated with and without fusion. RESULTS: The JOA recovery rate at final follow-up did not differ significantly between the fusion (n = 17, including all 15 patients with atlantoaxial subluxation) and non-fusion (n = 12) groups. However, pseudotumor regression was significantly more frequent in the fusion group (100% vs. 42%, p < 0.01). In all patients, regression cases had significantly higher rates of contrast enhancement of the pseudotumor on pre-operative T1 gadolinium-enhanced MRI (68% vs. 14%, p = 0.013) and of JOA recovery (50% vs. 30%, p < 0.01). CONCLUSIONS: Regression of pseudotumor occurred in all cases treated with fusion surgery. There was a significant difference in pseudotumor regression with or without fusion, and regression was significantly related to gadolinium enhancement on MRI. Therefore, it is preferable to use fusion surgery for a retro-odontoid pseudotumor that shows contrast enhancement, even if there is no apparent instability pre-operatively. These slides can be retrieved under Electronic Supplementary Material.