A new etiology of nontraumatic C0-C1-C2-Complex instability - abnormality of musculus rectus capitis posterior minor: a case report.

PURPOSE: This study presents an abnormality of the musculus rectus capitis posterior minor (RCPmi) as a new etiological factor for nontraumatic sagittal plane instability in the C0-C1-C2-complex, with a focus on identifying the absence or atrophy of RCPmi on both sides. METHODS: A 36-year-old male patient presented with recurring neck pain (VAS 8/10) and tingling paresthesia in the entire left hand over a six-month period, without significant neurological deficits. Radiated arm pain was not reported. Imaging examinations revealed sagittal plane instability in the C0-C1-C2-complex, spinal canal stenosis (SCS), and myelopathy at the C1 level. Subsequently, a dorsal C0-1 reposition and fusion with laminectomy were performed. RESULTS: The congenital absence or atrophy of RCPmi, leading to the lack of cephalad-rearward traction on the C1-tuberculum-posterius, induced a developmental failure of the C1 posterior arch. Consequently, the oblate-shaped C1 posterior arch lost support from the underlying C2 posterior arch and the necessary cephalad-rearward traction throughout the patient's 36-year life. This gradual loss of support and traction caused the C1 posterior arch to shift gradually to the anterior side of the C2 posterior arch, resulting in a rotational subluxation centered on the C0/1 joints in the sagittal plane. Ultimately, this led to SCS and myelopathy. Traumatic factors were ruled out from birth to the present, and typical degenerative changes were not found in the upper cervical spine, neck muscles, and ligaments. CONCLUSION: In this case, we not only report the atrophy or absence of RCPmi as a new etiological factor for nontraumatic sagittal plane instability in the C0-C1-C2-complex but also discovered a new function of RCPmi. The cephalad-rearward traction exerted by RCPmi on the C1 posterior arch is essential for the development of a normal C1 anterior-posterior diameter.

Relationship between clinical parameters and malformations in dogs diagnosed with atlanto-axial instability.

Atlanto-axial instability is a common disease that affects toy-breed dogs. Most cases of atlanto-axial instability are congenital. Furthermore, patients with atlanto-axial instability are predisposed to other concurrent diseases. Therefore, this study aimed to retrospectively determine the presence of concurrent diseases in cases with atlanto-axial instability using imaging data and analyze the relationship between clinical parameters and the incidence of complex malformations. The clinical data and imaging findings of 41 toy-breed dogs diagnosed with atlanto-axial instability were analyzed using their medical records and imaging data. Occipital dysplasia (17/27), atlanto-occipital overlapping (22/34), dens dysplasia (27/41), Chiari-like malformation (8/34), syringomyelia (5/34), lateral ventricular enlargement (20/36), and intracranial arachnoid cyst (5/35) were observed in patients with atlanto-axial instability. The body weight of the patients in the groups with atlanto-occipital overlapping and lateral ventricular enlargement was lower than that of those in the groups without these diseases (1.78 ± 0.71 vs 2.71 ± 1.15 kg, P = 0.0269, 1.60 ± 0.40 vs 2.75 ± 1.08 kg, P = 0.001, respectively). Furthermore, when the correlation between the total number of concurrent diseases and the age at onset and body weight was examined, it became clear that lower body weight was associated with the incidence of a greater number of concurrent diseases. Thus, the findings of this study suggest that toy-breed dogs are more likely to present with complex malformations and should be evaluated carefully with additional examinations and treatment methods.

Early Outcomes of Bone Autografting in the Bilateral Atlantoaxial Joints Applied in Posterior Fusion Surgery.

OBJECTIVE: Cable-dragged reduction and cantilever beam internal fixation can provide promising results in the treatment of atlantoaxial dislocation or instability. However, bilateral atlantoaxial joints bone autografting has not been conducted in this technique. We aim to evaluate the safety and effectiveness of bilateral atlantoaxial joints bone autografting in posterior cable-dragged reduction and cantilever-beam internal fixation. METHODS: In this retrospective study, we included 14 patients with a minimum 24-month follow-up from December 2019 to September 2020. The granular bone harvested from the iliac crest was packed into the bilateral atlantoaxial joints of 14 patients in posterior cable-dragged reduction and cantilever-beam internal fixation. X-ray imaging and cervical computed tomography (CT) were performed during follow-up. The time required for bone fusion was recorded. The clinical outcomes were evaluated using the JOA scores, NDI, and VAS scores. Mann-Whitney U test, the chi-squared test, or the Fisher exact test were used to compare the two groups regarding patient characteristics, clinical outcomes, bone fusion rates, and cervical sagittal alignment. RESULTS: The operations were successfully performed in all patients without any intraoperative complications. The mean operation time was (169.64 ± 20.91) minutes, and the intraoperative blood loss was (130.71 ± 33.62) mL. All patients received satisfactory reductions and firm bony fusion at the final follow-up. The fusion rates were 64.29% in the atlantoaxial joints and 21.43% in post bone graft area at 3 months postoperatively, and a significant difference was observed (p = 0.022). Besides, the cervical sagittal alignment in all patients was well maintained in the last follow-up compared to preoperatively. Importantly, a complete bony fusion in the atlantoaxial joints was observed in all patients. Moreover, the JOA, NDI, and VAS scores had improved significantly at the last follow-up. CONCLUSION: Bone autografting of the bilateral atlantoaxial joints is a safe and effective technique to increase bone fusion rates, shorten bone fusion time, and reduce complication rates when the cable-dragged reduction and cantilever beam internal fixation approach is used. Therefore, it is a cost-effective surgical procedure for treating patients with atlantoaxial dislocation or instability.

Craniocervical fusion in the paediatric population - case series of 21 patients.

PURPOSE: Craniocervical junction instability in a paediatric population presents a formidable challenge to the neurosurgeon. With a variety of anatomical variations, diminutive bony and ligamentous structures, possible superimposed syndromic abnormalities, the craniocervical junction is a technically challenging region to operate within. We aimed to review our series of patients to identify the common pathologies necessitating craniocervical fusion along with the use of intraoperative image guidance. METHODS: We performed a retrospective review of twenty-one patients, with a mean age of 8.1 years, undergoing craniocervical fixations, involving either occipitocervical fusion or atlantoaxial fixation, from a single institution over a twelve-year period. The presentation, preoperative investigations, indication for surgery, surgical procedure, use of intraoperative navigation and clinical and radiological results were examined. RESULTS: Twelve patients underwent primary C1-2 fixation, with the remaining 9 undergoing occipitocervical fusion. Five patients underwent surgery for traumatic instability, seven for os odontoideum, six for congenital anomalies and three for post-infectious instability. Follow up for 20 patients averaged 34 months (range 2-93 months). Five patients had Trisomy 21 and all underwent C1-2 fixation. Frameless stereotactic image guidance was utilised in five patients. No patients suffered immediate complications resulting from craniocervical junction fusion. Patients were observed to either neurologically improve (n = 6), or arrest their neurological deterioration following surgical intervention (n = 14), with concomitant radiological evidence of osseous fusion at follow up in 18 of 20 patients (90%). Two patients (10%) had radiological evidence of screw lucency, but neither required intervention because of being asymptomatic. CONCLUSIONS: Craniocervical fixation in a paediatric population is a viable and safe treatment modality for the management of disorders requiring stabilisation at the craniocervical junction. Our experience of utilising frameless stereotaxy in the setting of grossly distorted anatomical landmarks is also reported.

Myelopathy resulting from degenerative atlantoaxial subluxation.

PURPOSE: To present the clinical features and treatment strategy of degenerative atlantoaxial subluxation (DAAS). METHODS: Patients with DAAS treated in our institution from 2003 to 2020 were retrospectively reviewed. We utilized the Japanese Orthopedic Association (JOA) scale to evaluate the neurologic status and distance of Ranawat et al. (DOR) to measure vertical migration. RESULTS: We recruited 40 patients with > 2 years of follow-up and an average age of 62.3 ± 7.7 years. All the patients had myelopathy; only one patient had moderate trauma before exacerbation of symptoms, and the duration of symptoms was 34 ± 36 months. The most frequent radiological features were vertical migration of C1 (100%), sclerosis (100%), and narrowing of the atlantoaxial lateral mass articulations (100%). Two patients underwent transoral release combined with posterior reduction and fusion, and 38 patients underwent posterior reduction and fusion with C1 lateral mass screws-C2 pedicle screws and plate systems only. Forty cases (100%) achieved a solid atlantoaxial fusion, and 38 cases (95%) achieved anatomic atlantoaxial reduction. The JOA score increased from 9.3 ± 2.6 to 14.8 ± 2.1 (P < 0.01). DOR increased from 14.5 ± 2.5 to 17.8 ± 2.2 mm at the final follow-up (P < 0.01). Loosening of the locking caps was detected in one case, bony fusion was achieved, and harvest-site pain was reported in five patients. CONCLUSION: DAAS differs from other types of AAS and presents with anterior subluxation combined with vertical subluxation arising from degenerative changes in the atlantoaxial joints. We recommend anatomic reduction as an optimal strategy for DAAS.

Study on articular surface morphology of atlantoaxial lateral mass based on differential manifold.

OBJECTIVES: To propose a surface reconstruction algorithm based on a differential manifold (a space with local Euclidean space properties), which can be used for processing of clinical images and for modeling of the atlantoaxial joint. To describe the ideal anatomy of the lateral atlantoaxial articular surface by measuring the anatomical data. METHODS: Computed tomography data of 80 healthy subjects who underwent cervical spine examinations at our institution were collected between October 2019 and June 2022, including 46 males and 34 females, aged 37.8 ± 5.1 years (28-59 years). A differential manifold surface reconstruction algorithm was used to generate the model based on DICOM data derived by Vision PACS system. The lateral mass articular surface was measured and compared in terms of its sagittal diameter, transverse diameter, articular surface area, articular curvature and joint space height. RESULTS: There was no statistically significant difference between left and right sides of the measured data in normal adults (P > 0.05). The atlantoaxial articular surface sagittal diameter length was (15.83 ± 1.85) and (16.22 ± 1.57) mm on average, respectively. The transverse diameter length of the articular surface was (16.29 ± 2.16) and (16.49 ± 1.84) mm. The lateral articular surface area was (166.53 ± 7.69) and (174.48 ± 6.73) mm2 and the curvature was (164.03 ± 5.27) and (153.23 ± 9.03)°, respectively. The joint space height was 3.05 ± 0.11mm, respectively. There is an irregular articular space in the lateral mass of atlantoaxial, and both upper and lower surfaces of the articular space are concave. A sagittal plane view shows that the inferior articular surface of the atlas is mainly concave above; however, the superior articular surface of the axis is mainly convex above. In the coronal plane, the inferior articular surface of the atlas is mostly concave above, with most concave vertices located in the medial region, and the superior articular surface of the axis is mainly concave below, with most convex vertices located centrally and laterally. CONCLUSION: A differential manifold algorithm can effectively process atlantoaxial imaging data, fit and control mesh topology, and reconstruct curved surfaces to meet clinical measurement applications with high accuracy and efficiency; the articular surface of the lateral mass of atlantoaxial mass in normal adults has relatively constant sagittal diameter, transverse diameter and area. The distance difference between joint spaces is small, but the shape difference of articular surfaces differs greatly.

C2 Odontoid Fracture Associated with C1-C2 Rotatory Dislocation: A Retrospective Analysis of 2 Surgical Techniques.

BACKGROUND: Odontoid fractures in association with a C1-C2 rotatory luxation reports are seldom found in the literature. The fusion between the lateral mass of C1 and C2 could be of interest to ensure adequate treatment in these particular cases. We report 23 cases where there was coexistence of an odontoid fracture and rotatory subluxation, which were treated surgically using cages between C1 and C2 or just traditional Goel-Harms technique. We evaluated the radiologic fusion rate, reoperation rate, and complications. METHODS: This was a single-center, retrospective, cohort study of patients with C2 fractures (mixed type and C1-C2 rotatory luxation according to the Fielding classification) who were treated surgically. Radiologic computed tomography scans were used to assess fusion (presence of bridging trabecular bone end plate or pseudoarthrosis) between 6 months and 1.5 years after the surgery. RESULTS: Twenty-three patients were diagnosed with C2 fractures and C1-C2 rotatory luxation that were treated surgically and were suitable for the analysis; 11 patients underwent C1-C2 fusion with intra-articular cages, and 12 underwent a classical Goel-Harms technique. The fusion rate at the C1-C2 joint was higher in the cages group. Only 12 patients exhibited fusion at the level of the odontoid fracture. CONCLUSIONS: C2 fractures associated with C1-C2 rotatory dislocation are rare. The fusion rate at the level of the odontoid in these patients appears to be lower than that reported in patients without rotatory dislocation. It may be of special interest to obtain a clear fusion at the C1-C2 joint, where this type of implant seems to offer an advantage.

Management of the Vertebral Artery in Craniovertebral Junction Stabilization Surgery.

The vertebral artery (VA) has an intimate relationship with the bones of the craniovertebral junction. An exact understanding of the VA anatomy in general and in the specific surgical case in particular is absolutely necessary in order to avoid intraoperative vascular injury. The course of the VA on the inferior aspect of the superior facet of the C2 vertebra makes it susceptible to damage during transarticular and interarticular fixation with the screw insertion in the adjacent lateral mass. The consequences of the intraoperative VA injury will depend on the patency of other arteries supplying the brain. In case of this complication, quick decision-making is essential to avoid excessive blood loss and to preserve adequate cerebral blood flow.

Biomechanical properties of different anterior and posterior techniques for atlantoaxial fixation: a finite element analysis.

BACKGROUND: Many techniques for atlantoaxial fixation have been developed. However, the biomechanical differences among various atlantoaxial fixation methods remain unclear. This study aimed to evaluate the biomechanical influence of anterior and posterior atlantoaxial fixation techniques on fixed and nonfixed segments. METHODS: An occiput-C7 cervical finite element model was used to construct 6 surgical models including a Harms plate, a transoral atlantoaxial reduction plate (TARP), an anterior transarticular screw (ATS), a Magerl screw, a posterior screw-plate, and a screw-rod system. Range of motion (ROM), facet joint force (FJF), disc stress, screw stress, and bone-screw interface stress were calculated. RESULTS: The C1/2 ROMs were relatively small in the ATS and Magerl screw models under all loading directions except for extension (0.1°-1.0°). The posterior screw-plate system and screw-rod system generated greater stresses on the screws (77.6-1018.1 MPa) and bone-screw interfaces (58.3-499.0 MPa). The Harms plate and TARP models had relatively small ROMs (3.2°-17.6°), disc stress (1.3-7.6 MPa), and FJF (3.3-106.8 N) at the nonfixed segments. Changes in disc stress and FJF of the cervical segments were not consistent with changes in ROM. CONCLUSIONS: ATS and Magerl screws may provide good atlantoaxial stability. The posterior screw-rod system and screw-plate system may have higher risks of screw loosening and breakage. The Harms plate and TARP model may more effectively relieve nonfixed segment degeneration than other techniques. The C0/1 or C2/3 segment may not be more susceptible to degeneration than other nonfixed segments after C1/2 fixation.

Dynamics of atlantoaxial rotation related to age and sex: a cross-sectional study of 308 subjects.

BACKGROUND CONTEXT: Physiological ranges and dynamic changes of atlantoaxial rotation (ROTC1/2), total cervical spine rotation (ROTCs) and the percentage of ROTC1/2 from ROTCs (ROTCperc) for different age groups have not yet been investigated in a sufficiently sized cohort. Furthermore, it is not clear whether demographic variables such a sex, smoking status or diabetes affect ROTC1/2, ROTCs and ROTCperc. PURPOSE: Obtain physiological ranges of ROTC1/2, ROTCs and ROTCperc for different age groups and determine their age-based dynamics. Investigate whether ROTC1/2, ROTCs and ROTCperc are affected by sex, smoking status or diabetes. DESIGN: Observational cross-sectional study. PATIENT SAMPLE: Patients undergoing elective CT examinations of the head and neck region between August 2020 and January 2022. OUTCOME MEASURES: Ranges of motion of ROTC1/2, ROTCs and ROTCperc in degrees. METHODS: A total of 308 subjects underwent dynamic rotational CT examinations of the upper cervical spine. Patients were divided into three age categories A1 (27-49 years), A2 (50-69 years) and A3 (≥70 years). Category A3 was further divided into B1 (70-79 years) and B2 (≥80 years). Values of ROTC1/2, ROTCs and ROTCperc were compared between all age groups, males and females, smokers and nonsmokers, diabetics a nondiabetics. Dynamics of ROTC1/2, ROTCs related to age and sex were visualized using scatterplot and trendline models. RESULTS: ROTC1/2 significantly decreased from group A1 (64.4°) to B2 (46.7°) as did ROTCs from A1 (131.2°) to B2 (97.6°). No significant differences of ROTperc were found between groups A1-B2 with values oscillating between 49% and 51%. Smoking and diabetes did not significantly affect ROTC1/2, ROTCs and ROTCperc, females had significantly higher ROTCs than males. Males and females demonstrated a different dynamic of ROTC1/2 and ROTCs demonstrated by out scatterplot and trendline models. CONCLUSIONS: Both ROTC1/2 and ROTCs significantly decrease with age, whereas ROTCperc remains stable. Females demonstrated higher ROTCs and their decrease of ROTC1/2 and ROTCs occurred in higher age groups compared to males. The functional repercussions atlantoaxial fusion are variable based on patient age and sex and should be taken into account prior to surgery.

Correlation Among Syrinx Resolution, Cervical Sagittal Realignment, and Surgical Outcome After Posterior Reduction for Basilar Invagination, Atlantoaxial Dislocation, and Syringomyelia.

BACKGROUND: The correlation among syrinx resolution, occipitoaxial sagittal alignment, and surgical outcome in long-term follow-up seems to have not been clarified. OBJECTIVE: To further explore the relationship between the syrinx resolution and occipitoaxial realignment after posterior reduction and fixation in basilar invagination (BI)-atlantoaxial dislocation (AAD) patients with syringomyelia. METHODS: A continuous series of 32 patients with BI-AAD and syringomyelia who received direct posterior reduction met the inclusion criteria of this study. Their clinical and imaging data were analyzed retrospectively. Before surgery and at the last follow-up, we used the Japanese Orthopedic Association (JOA) score and the Neck Disability Index (NDI) to assess the neurological status, respectively. The Pearson correlation coefficient and multiple stepwise regression analysis were used to explore the relevant factors that may affect surgical outcomes. RESULTS: There were significant differences in atlantodental interval, clivus-axial angle, occiput-C2 angle (Oc-C2A), cervicomedullary angle (CMA), subarachnoid space (SAS) at the foramen magnum (FM), syrinx size, NDI, and JOA score after surgery compared with those before surgery. ΔCMA and the resolution rate of syrinx/cord as relevant factors were correlated with the recovery rate of JOA (R 2 = 0.578, P < .001) and NDI (R 2 = 0.369, P < .01). What's more, ΔSAS/FMD (SAS/FM diameter) and ΔOc-C2A were positively correlated with the resolution rate of syrinx/cord (R 2 = 0.643, P < .001). CONCLUSION: With medulla decompression and occipital-cervical sagittal realignment after posterior reduction and fusion for BI-AAD patients with syringomyelia, the structural remodeling of the craniovertebral junction and occipitoaxial realignment could contribute to syringomyelia resolution.

Posterior Atlantoaxial Fusion With C1-2 Pedicle Screw Fixation for Atlantoaxial Dislocation in Pediatric Patients With Mucopolysaccharidosis IVA (Morquio a Syndrome): A Case Series.

OBJECTIVE: To investigate the efficacy and safety of posterior atlantoaxial fusion (AAF) with C1-2 pedicle screw fixation for atlantoaxial dislocation (AAD) in pediatric patients with mucopolysaccharidosis IVA (MPS IVA). METHODS: This study included 21 pediatric patients with MPS IVA who underwent posterior AAF with C1-2 pedicle screw fixation. Anatomical parameters of the C1 and C2 pedicle were measured on preoperative computed tomography (CT). The American Spinal Injury Association (ASIA) scale was used to evaluate the neurological status. The fusion and accuracy of pedicle screw was assessed on postoperative CT. Demographic, radiation dose, bone density, surgical, and clinical data were recorded. RESULTS: Patients reviewed included 21 patients younger than 16 years with an average age of 7.4 ± 4.2 years and an average of 20.9 ± 7.7 months follow-up. Fixation of 83 C1 and C2 pedicle screws was performed successfully and 96.3% of them were identified as being safe. One patient developed postoperative transient disturbance of consciousness and one developed fetal airway obstruction and died about 1 month after the surgery. Out of the remaining20 patients, fusion was achieved, symptoms were improved, and no other serious surgical complications were observed at the latest follow-up. CONCLUSIONS: Posterior AAF with C1-2 pedicle screw fixation is effective and safe for AAD in pediatric patients with MPS IVA. However, the procedure is technically demanding and should be performed by experienced surgeons with strict multidisciplinary consultations.

Surgical treatment of craniovertebral junction instability in children with Down syndrome: a systematic review.

OBJECTIVE: Down syndrome (DS) affects 1 in 700 live births and approximately one-third of patients develop craniovertebral junction (CVJ) instability, diagnosed by clinical examination and radiological measures such as the atlantodens interval (ADI) and space available for the cord (SAC). Patients with symptomatic CVJ instability are at increased risk for spinal cord injury. There are no guidelines for surgical management of CVJ instability in DS, the existing literature is sparse, and there is a lack of consistent pediatric data. This systematic review aimed to synthesize practice patterns of the surgical management of CVJ stability in pediatric DS patients to facilitate future standardization of care. METHODS: Peer-reviewed studies reporting surgical management of CVJ instability in pediatric DS patients were systematically reviewed. Inclusion criteria were studies reporting primary data on patients younger than 18 years with DS, who had CVJ instability evaluation and underwent surgical treatment. Bias risk was assessed. Descriptive statistics of the independent patient data were presented. Interval variables were analyzed using the Wilcoxon rank-sum test. RESULTS: Of 1056 records, 38 studies were included. Of the included patients, 169 (6%) underwent surgery. The surgical indication was symptomatic, radiologically confirmed CVJ instability in 81% of the patients, presenting with myelopathy (30%), weakness (25%), abnormal gait (24%), torticollis (15%), and neck pain (14%). A cutoff of ADI ≥ 4 mm or SAC ≤ 14 mm, cord compression, cord signal change, and anomalous bony anatomy were used in diagnosing CVJ instability. Surgical approaches focused on internal fixation with posterior occipitocervical or atlantoaxial instrumented fusion in 57% and 44% of patients, respectively. Autograft, wiring, and allograft constructs were used in 48%, 45%, and 9% of patients. Anterior cervical approaches were performed in 6% of patients. Preoperative and postoperative external orthoses were used in approximately 50% of patients. The surgical mortality rate was 3%, and the complication rate was 36%. CONCLUSIONS: Assessment of CVJ instability in DS is based on radiographic and clinical factors. Surgery is recommended if symptoms are present, and the procedure type depends on patient factors, degree of instability, anomalous bony anatomy, and reduction results to relieve cord compression. Most commonly, posterior instrumented fusion is used. However, further research is required to determine the strength of evaluation methods, create standardized guidelines for evaluation and surgical treatment, and investigate the long-term results of different surgical techniques.

Predictors factors of atlanto-axial subluxation in Tunisian patients with rheumatoid arthritis.

BACKGROUND: The aim of this study was to describe clinical and imaging features of atlantoaxial subluxation (AAS) and the associated risk factors in patients with rheumatoid arthritis (RA). METHODS: We conducted a retrospective and comparative study including 51 RA patients with AAS and 51 RA patients without AAS. Atlantoaxial subluxation was defined by the presence of an anterior C1C2 diastasis on the cervical spine radiograph in hyperflexion and/or an anterior, posterior, lateral or rotatory C1C2 dislocation on MRI with/without inflammatory signal. RESULTS: In G1, clinical presentations revealing AAS were mainly neck pain (68.7%) and neck stiffness (29.8%). MRI revealed: diastasis C1C2 (92.5%), periodontoid pannus (92.5%), odontoid erosion (23.5%), vertical subluxation (9.8%) and spinal cord involvement (7.8%). A collar immobilisation and corticosteroid boluses were indicated in 86.3% and 47.1% of cases. C1-C2arthrodesis was performed in 15.4% of cases. Atlantoaxial subluxation was significantly associated with: age at disease onset (p = 0.009), history of joint surgery (p = 0.012), disease duration (p = 0.001), rheumatoid factor (p = 0.01), anti-cyclic citrullinated peptide (p = 0.02), erosive radiographic status (p < 0.005), coxitis (p < 0.001), osteoporosis (p = 0.012), extra-articular manifestations (p < 0.001), and high disease activity (p = 0.001). Multivariate analysis identified RA duration (p < 0.001, OR = 1.022 CI[1.01-1.034]) and erosive radiographic status (p = 0.01, OR = 21.236 CI[2.05-219.44]) as predictive factors of AAS. CONCLUSION: Our study showed that longer disease duration and joint destruction are the major predictive factors of AAS. Early treatment initiation, tight-control and regular monitoring of cervical spine involvement are required in these patients.

Sagittal Reconstruction of the Atlantoaxial Lateral Mass Complex with an Intra-Articular Cage Fusion Technique for Degenerative Atlantoaxial Instability.

OBJECTIVE: To evaluate outcomes of sagittal reconstruction of the atlantoaxial lateral mass complex using a modified intra-articular cage fusion technique for treating degenerative atlantoaxial instability. METHODS: Data from 15 patients with degenerative atlantoaxial instability were retrospectively reviewed. All patients underwent posterior reduction and intra-articular fusion with a cage filled with local autologous bone. Atlantodental interval values on plain radiography in flexion before and after surgery were recorded. Bone fusion was evaluated on computed tomography reconstruction, and bone fusion time was recorded. Lateral atlantoaxial joint space height before and after surgery was measured on coronal computed tomography reconstruction. Japanese Orthopaedic Association score and visual analog scale score for neck pain before surgery and at final follow-up were compared. RESULTS: Mean follow-up time was 40.7 ± 13.4 months. All patients achieved good reduction and solid bone fusion at follow-up. Mean fusion time was 4.4 ± 1.1 months. Atlantodental interval decreased from 8.6 ± 1.5 mm preoperatively to 1.9 ± 0.5 mm at final follow-up (P < 0.05). Lateral atlantoaxial joint space height significantly improved from 1.7 ± 0.5 mm preoperatively to 4.7 ± 0.3 mm at final follow-up (P < 0.05). Japanese Orthopaedic Association score significantly improved from 14.9 ± 1.5 preoperatively to 16.7 ± 0.6 at final follow-up (P < 0.05). Visual analog scale score for neck pain markedly decreased from 4.5 ± 1.8 preoperatively to 0.5 ± 0.6 at final follow-up (P < 0.05). CONCLUSIONS: Posterior reduction and intra-articular cage fusion with a C2 nerve root preservation technique is effective in treatment of degenerative atlantoaxial instability. Satisfactory reconstruction of the sagittal alignment and the height of atlantoaxial complex can be achieved.

Congenital atlanto-occipital dislocation in a patient with Down syndrome: a case report.

Down syndrome, also known as trisomy 21, is associated with congenital cervical spine abnormalities, including atlantoaxial instability with or without os odontoideum, atlanto-occipital instability, and hypoplasia of the atlas. Herein, we report a case of Down syndrome complicated by congenital atlanto-occipital dislocation. The patient presented with severe cervical myelopathy at 13 years of age after a 10-year follow-up. Radiography and computed tomography revealed os odontoideum protruding into the foramen magnum and congenital anterior atlanto-occipital dislocation. Additionally, a bifurcated internal occipital crest with a thinned central portion of the occipital bone was noted. Magnetic resonance imaging revealed kyphotic alignment of the spinal cord with severe compression at the foramen magnum level. As the neurological impairment was partially improved by halo vest immobilization, we performed in situ O-C2 fusion with an iliac autograft and decompression of the foramen magnum and posterior arch of C1. An improvement was observed immediately after surgery. Two years after surgery, radiography and computed tomography showed solid O-C2 segment fusion. The accumulation of similar cases is essential for determining the prognosis or optimal treatment for this rare congenital condition.

Clinical and Surgical Characteristics of Patients with Atlantoaxial Dislocation in the Setting of Sandwich Fusion: A Case-Control Analysis of Over 500 Patients with Mid-Term to Long-Term Follow-up.

BACKGROUND: Patients with "sandwich" fusion (concomitant C1 occipitalization and C2-C3 nonsegmentation), a subtype of Klippel-Feil syndrome, are at particular risk for developing atlantoaxial dislocation (AAD). However, the clinical and surgical characteristics of AAD in patients with sandwich fusion have not been clearly defined. METHODS: A retrospective case-control study with a large sample size and a minimum 2-year follow-up was performed. From 2000 to 2018, 253 patients with sandwich AAD underwent a surgical procedure; these patients constituted the case group, and a matching number of patients with non-sandwich AAD were randomly selected to form the control group. Clinical data from electronic medical records and various imaging studies were analyzed and compared. The Japanese Orthopaedic Association (JOA) scale was used to evaluate neurological function. RESULTS: Patients with sandwich AAD, compared with patients with non-sandwich AAD, had symptom onset at a younger age (34.8 compared with 42.8 years; p < 0.001) and had a higher likelihood for myelopathy (87.4% compared with 74.7%; p < 0.001). Patients with sandwich AAD had a higher incidence of lower cranial nerve palsy (7.9% compared with 0.0%; p < 0.001), a lower preoperative JOA score (13.4 compared with 14.2; p < 0.001), and higher incidences of accompanying Type-I Chiari malformation (20.9% compared with 1.2%; p < 0.001) and syringomyelia (21.3% compared with 1.6%; p < 0.001). Finally, patients with sandwich AAD had higher likelihoods of undergoing transoral release (28.5% compared with 5.1%; p < 0.001) and use of salvage fixation techniques (34.4% compared with 6.3%; p < 0.001), and had lower postoperative results for the JOA score (14.9 compared with 15.9; p < 0.001) and improvement rate (43.8% compared with 58.2%; p < 0.001). CONCLUSIONS: Patients with sandwich AAD demonstrated distinct clinical manifestations. Versatility involving the use of various internal fixation techniques and transoral release procedures was frequently required in the surgical management of these patients, and meticulous and personalized preoperative planning would be of paramount importance. LEVEL OF EVIDENCE: Prognostic Level III . See Instructions for Authors for a complete description of levels of evidence.

[Atlantoaxial rotatory subluxation - a cause of torticollis in children]. / Atlantoaxial subluxation kan vara en orsak till tortikollis hos barn.

Atlantoaxial rotatory subluxation is an important differential diagnosis in young children with torticollis. The condition rarely results in neurological deficits. Radiological examinations such as computer tomography (CT) and magnetic resonance imaging (MRI) are useful to determine the diagnosis. It is important for doctors in all areas of healthcare to be aware of the condition, as persistent problems or surgery can be avoided by early detection and treatment.

Treating Pediatric Irreducible Atlantoaxial Rotatory Fixation (IAARF) by Unlocking Facet Joint Through Transoral Approach and Fixing With Slim-TARP Plate (15 Cases Series).

BACKGROUND: Irreducible atlantoaxial rotatory fixation (IAARF) often requires surgical treatment. Transoral unlocking the facet joints is a key measure for the treatment of IAARF. We investigate a novel method for treating pediatric IAARF by unlocking facet joint through transoral appraoch and fixed with slim-tarp plate in same stage with same approach. OBJECTIVE: The objective of this study is to investigate the method and efficacy of a unique transoral approach to unlock facet joints and fixation with slim-shaped transoral anterior reduction plate (slim-TARP) plate in the treatment of IAARF. METHODS: Fifteen patients diagnosed with AARF were transferred to our hospital. After 1 week of bidirectional cervical cranial traction, they were diagnosed with irreducible AARF that, and then underwent transoral release and fixation with slim-TARP plate procedures. Postoperative computed tomography and magnetic resonance were used to evaluate the reduction effect, bone fusion, and fusion time. Japanese orthopaedic association scores were used to compare the recovery of spinal cord function in patients before and after surgery. Complications such as wound infection, neurovascular injury, and loosening of internal fixation were evaluated too. RESULTS: All 15 patients underwent transoral unlocking facet joint and fixation with slim-TARP procedures smoothly. The operation time were 129.2±11.9 minutes, blood loose were 83±23 mL. There were no neurological injury, wound infections, verified or suspected vertebral artery injury, etc. All patients were followed up for a mean of 17.8±6.6 months (range: 12 to 36 mo). Bony fusion was achieved in all patients. Mean fusion time was 3.6±1.2 months (range: 3 to 6 mo). Complete correction of torticollis was achieved in all 15 cases. Preoperative symptoms of neck pain and limitation of neck movement were effectively alleviated at 3 months after surgery. The 3 patients with preoperative neurological deficits had significant relief after surgery, and their latest follow-up results showed that their Japanese orthopaedic association scores increased from 13.0±1.0 to 16.3±0.6. CONCLUSIONS: Transoral release and fixation with slim-TARP plate by transoral approach is a feasible and safe method for treating pediatric irreducible atlantoaxial rotatory fixation.