Craniovertebral Junction Abnormalities Research Articles

Are Chiari malformation and basilar invagination associated with jugular foramen stenosis?

ObjectiveTo compare the diameter of the jugular foramen (JF) between normal individuals and those with the diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI). MethodsThis cross-sectional analytical study measured the diameter of right and left JF in magnetic resonance imaging of patients that were split into four groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, while univariate ANOVA with Tukey's post-hoc test assessed JF differences between groups. ResultsConsidering all combinations of laterality and sex, CMI+BI had JF narrower than their respective controls. The JF was bilaterally narrower in BI than the control, only in females. Regardless of laterality, CMI had wider JF than the CMI+BI in males, and it did not differ from the control. In the females of CMI, the right JF did not differ from the control, and it was wider than the CMI+BI; while the left JF was narrower than the control, and it did not differ from the CMI+BI. ConclusionJF narrowing is associated with CMI and BI when they are found combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claims a possible role of intracranial pressure disturbances as a triggering factor for CMI.

Natural history of craniovertebral abnormalities in a single-center study in 54 patients with Hurler syndrome.

Craniovertebral junction (CVJ) abnormalities are common and well documented in mucopolysaccharidosis type I-Hurler syndrome (MPS IH), often causing severe spinal canal narrowing. However, the requirement for surgical decompression and/or fusion is uncommon. Although hematopoietic cell transplant (HCT) has been shown to prolong the lives of patients with MPS IH, its effect in halting or reversing musculoskeletal abnormalities is less clear. Unfortunately, there are currently no universal guidelines for imaging or indication for surgical interventions in these patients. The goal of this study was to track the progression of the CVJ anatomy in patients with MPS IH following HCT, and to examine radiographic features in patients who needed surgical intervention. Patients with MPS IH treated at the University of Minnesota with allogeneic HCT between 2008 and 2020 were retrospectively reviewed. Patients who underwent CVJ surgery were identified with chart review. All MPS IH cervical scans were examined, and the odontoid retroflexion angle, clivoaxial angle (CXA), canal width, and Grabb-Oakes distance (pB-C2) were measured yearly for up to 7 years after HCT. Longitudinal models based on the measurements were made. An intraclass correlation coefficient was used to measure interrater reliability. Nine children without MPS IH were examined for control CVJ measurements. A total of 253 cervical spine MRI scans were reviewed in 54 patients with MPS IH. Only 4 (7.4%) patients in the study cohort required surgery. Three of them had posterior fossa and C1 decompression, and 1 had a C1-2 fusion. There was no statistically significant difference in the spinal parameters that were examined between surgery and nonsurgery groups. Among the measurements, canal width and CXA varied drastically in patients with different neck positions. Odontoid retroflexion angle and CXA tended to decrease with age. Canal width and pB-C2 tended to increase with age. Based on the data, the authors observed an increase in canal width and pB-C2, whereas the CXA and odontoid retroflexion angle became more acute as the patients aged after HCT. The longitudinal models derived from these data mirrored the development in children without MPS IH. Spinal measurements obtained on MR images alone are not sufficient in identifying patients who require surgical intervention. Symptom monitoring and clinical examination, as well as pathological spinal cord changes on MRI, are more crucial in assessing the need for surgery than is obtaining serial imaging.

Functional and radiological outcomes following craniovertebral junction surgery.

Craniovertebral junction (CVJ) pathologies include atlantoaxial instability/deformities resulting in myelopathy, respiratory failure, and even death. Here, we describe the indications, preoperative planning, and intra-operative/postoperative complications following surgical management of CVJ anomalies. A prospective analysis of 34 patients with CVJ pathology was evaluated between 2015 and 2022. Their various etiologies included atlantoaxial instability, trauma, tuberculosis, Down's syndrome, Morquio syndrome, os odontoideum, and atlantoaxial abnormalities. Clinical outcomes were assessed using the American spinal injury association (ASIA) impairment scale score and Benzel's modified Japanese Orthopedic Association (mJOA) score. Surgical assessments included length of hospital stay, operative time, blood loss, and intraoperative postoperative complications. Radiological parameters included fusion (i.e., implant loosening/implant failure), preoperative/ postoperative atlanto-dens interval (ADI), clivus canal angle (CCA), and space available for cord (SAC). Five patients were managed conservatively, while 29 patients had surgery. Operations included occipitocervical fusion (14 patients), C1-2 fusion (10 patients), C1-2 transarticular screw fixation (four patients), and one patient underwent anterior corpectomy decompression/fusion. Seven patients had vertebral artery anomalies, and 13 patients had atlantoaxial abnormalities. At the final follow-up, atlantoaxial instability (i.e., mean preoperative ADI of 6.6 ± 2.3 mm) was restored to 4.2 ± 0.6 mm, significant cord compression (i.e., with mean SAC of 8.3 ± 2.9 mm) was relieved to 17.2 ± 1.6 mm, and the mean preoperative CCA (i.e., 130.2 ± 15.3) was improved to 143.3 ± 8.3°. There was also a statistically significant improvement in the ASIA scale and mJOA score. Surgical management of CVJ abnormalities requires expertise and meticulous planning to avoid devastating complications such as wound dehiscence and catastrophic vertebral artery injury.

Management of Ventral Brainstem Compression in Chiari Malformation Type I.

Craniovertebral junction (CVJ) abnormalities are associated with the Chiari malformation type I. These abnormalities may lead to ventral brainstem compression which can be reduced with traction and posterior reduction using instrumentation. In other cases, the irreducible CVJ pathology with persistent ventral brainstem compression requires ventral decompression. In all cases, a posterior extradural or extra-intradural decompression is required along with an occipitocervical fusion to maintain the reduced and realigned CVJ or stabilize the CVJ after a ventral decompression.

Prediction of the functional and radiological outcome on the basis of independent factors with special emphasis on the use of 3D printed models in craniovertebral junction surgery.

Background: The aim of the study was to evaluate the advantage of performing planned surgery using customized three-dimensional (3D) printed models versus performing surgery without using 3D printed models in patients with craniovertebral junction (CVJ) anomalies and traumatic CVJ fractures and dislocations.Methods: Forty-two patients with CVJ anomalies, who were planned for operative intervention in the Department of Neurosurgery at SMS Hospital from March 2019 to February 2021, were randomly divided into two groups and analyzed. First group was operated after rehearsal on a customized 3D printed model whereas the second group underwent operative intervention without the rehearsal of surgery on the 3D printed model.Results: Forty-two patients were enrolled for the study. Twenty-five of these patients had developmental CVJ anomalies, 16 had post traumatic Atlantoaxial dislocation (AAD), and one had congenital AAD. Twenty-three patients underwent surgical intervention using 3D printed models and 19 without using 3D printed models. The outcome in the two groups was compared using modified Japanese orthopedic association score (mJOA), recovery rate, incidence of complications such as screw malposition, postoperative neurological deterioration, vertebral artery (VA) injury, and radiological improvement based on Atlanto-Dental interval, the distance of the tip of dens from Wackhenheims clivus canal line, and the distance of tip of dens from the Chamberlain’s line. The improvement in mJOA score postoperatively was found to be statistically significant in study group (P < 0.001) as compared to control group (P = 0.06). Recovery rate was better in study group than in control group (P = 0.023). In study group, the incidence of screw malposition and VA injury was lower than control group. Three patients deteriorated neurologically postoperatively in the control group and none in the study group. The average improvements in the radiological parameters were found to be better in study group as compared to control group postoperatively.Conclusion: The authors conclude that 3D printed models are extremely helpful in analyzing joints and VA anatomy preoperatively and are helpful in unmasking any abnormal bony and vascular anatomy effectively, making the surgeon confident about the placement of the screws intraoperatively. These 3D models help in intraoperative error minimization with better neurological outcomes in postoperative period. In our opinion, these models should be included as a basic investigation tool in patients of CVJ abnormalities. The models also offer other advantages such as preoperative simulation, teaching modules, and patient education.

Role of imaging in craniovertebral junction abnormalities

Background: Craniovertebral junction (CVJ) abnormalities constitute an important group of treatable neurological disorders with diagnostic dilemma. Their precise diagnosis and pre-treatment evaluation significantly affects prognosis and quality of life of patients. Aims: To localize, describe and characterize craniovertebral junction abnormalities, arrange frequently detected CVJ pathologic imaging findings and to emphasize clinical implications to improve our radiological report. Materials and methods: A Cross sectional study done for 24 months in 50 patients of all age groups with clinical suspicion of CVJ abnormalities.Results: In the acquired CVJ abnormality Trauma is the most common etiology(n=15) followed by Tuberculosis(n=4), Rheumatoid arthritis(n=3), Tumors(n=2). Most common age group involved was 2nd decade with male predominance. Most commonly presenting symptom is weakness of limbs followed by neck pain. Most common imaging abnormality is cervicomedullary junction compression and atlanto axial dislocation. ACM I and Syringomyelia are the commonest neural anomalies associated with bony CVJ anomalies. Most common congenital anomalies are atlanto axial dislocation followed by basilar invagination. Only one patient had syndromic association which was Klippel-feil syndrome. The commonest injuries involving the CVJ were odontoid fracture predominantly type II and the commonest combination of injury was C1 with odontoid fracture.

Efficacy of a Lateral Mass Fusion Device Combined with a Three-Dimensional–Printed Model in the Treatment of Craniovertebral Junction Abnormalities

To investigate the efficacy of a lateral mass fusion device combined with a three-dimensional-printed model in treatment of craniovertebral junction abnormalities. This retrospective study comprised 56 patients with irreducible atlantoaxial dislocation who underwent posterior fixation between January 2016 and December 2019. Patients were divided into 2 groups according to whether or not cages were used-cage group and autograft group. Visual analog scale score, Japanese Orthopaedic Association score, health-related quality of life, American Spinal Injury Association spinal cord injury grade, atlas-dens interval, space available for the cord, cervicomedullary angle, and fusion rate were compared between groups. Medical follow-up was >1 year. There was no statistical difference between groups in preoperative visual analog scale score, Japanese Orthopaedic Association score, 12-Item Short Form Health Survey score, American Spinal Injury Association grade, atlas-dens interval, space available for the cord, and cervicomedullary angle, and these indexes significantly improved after surgery (P < 0.05). Visual analog scale score and atlas-dens interval were lower in the cage group than in the autograft group (P < 0.05). Japanese Orthopaedic Association score, 12-Item Short Form Health Survey score, space available for the cord, and cervicomedullary angle were significantly higher in the cage group than in the autograft group (P < 0.05). Fusion rate of the cage group 4-6 months after surgery was higher than that of the autograft group (P= 0.068). American Spinal Injury Association grade was significantly higher in the cage group than in the autograft group (P < 0.05). During 1-year follow-up, neurological function improvement and atlantoaxial joint reduction were satisfactory. The lateral mass fusion device combined with a three-dimensional printed model may be a clinically useful technique.

Craniovertebral junction abnormalities in children: Surgical, anesthetic and intensive care implications

The craniovertebral junction is unique and the most complex of the axial skeleton in multiple aspects; embryology, anatomy, pathology and kinematics. A surgical physiological approach to management of its abnormalities was instituted at the University of Iowa Hospitals & Clinics (UIHC) in 1977 and has been the accepted treatment algorithm worldwide. A global referral led to a prospective database of more than 7,000 adult and pediatric patients; 3,600 of whom were treated at UIHC. 975 children (less than 18 years of age) were surgically managed. The commonest symptoms and signs were neck pain and headache, neurological deficit due to brain stem and spinal cord compression, vascular issues secondary to vertebral artery compression and the Chiari malformation.

Role of Three-dimensional Printing in Neurosurgery: An Institutional Experience.

Background:Recent advancements in three-dimensional (3D) printing technology in the field of neurosurgery have given a newer modality of management for patients. In this article, we intend to share our institutional experience regarding the use of 3D printing in three modalities, namely, cranioplasty using customized 3D-printed molds of polymethylmethacrylate, 3D-printed model-assisted management of craniovertebral (CV) junction abnormalities, and 3D model-assisted management of brain tumors.Materials and Methods:A total of 55 patients were included in our study between March 2017 and December 2019 at S. M. S Medical College, Jaipur, India. 3D-printed models were prepared for cranioplasty in 30 cases, CV junction anomalies in 18 cases, and brain tumors in 7 cases. Preoperative and postoperative data were analyzed as per the diagnosis.Results:In cranioplasty, cranial contour and approximation of the margins were excellent and esthetic appearance improved in all patients. In CV junction anomalies, neck pain and myelopathy were improved in all patients, as analyzed using the visual analog scale and the Japanese Orthopedic Association Scale score, respectively. Our questionnaire survey revealed that 3D models for brain tumors were useful in understanding space interval and depth intraoperatively with added advantage of patient education.Conclusion:Rapid prototyping 3D-printing technologies provide a practical and anatomically accurate means to produce patient-specific and disease-specific models. These models allow for surgical planning, training, simulation, and devices for the assessment and treatment of neurosurgical disease. Expansion of this technology in neurosurgery will serve practitioners, trainees, and patients.

BASILAR INVAGINATION: IMPORTANCE OF CRANIOMETRIC MEASUREMENT

<h3>BACKGROUND</h3> Basilar invagination (BI) is a congenital or degenerative craniovertebral junction abnormality that results from the odontoid process prolapsing into the foramen magnum. Even though the acquired form is frequently seen with rheumatoid arthritis, BI commonly presents in many congenital conditions such as in people with Chiari malformation. In another type, it has been classified as those in which the odontoid process invaginates into the foramen magnum, resulting in cranio-cervical instability (Type 1) and those with flattened base of cranium that is not associated with instability (Type 2). Clinical presentations include chronic headaches, limited neck motion, and acute neurologic deterioration. <h3>CASE REPORT</h3> A 31-year-old male patient presented for treatment of a sleep-related breathing disorder. Cone beam computed tomography (CBCT) was completed for airway evaluation. Standard radiographic parameters described by Chamberlain were used to assess the craniovertebral junction. Osseous fusion of the C1/C2 joint and extension of the odontoid process approximately 10.5 mm above the Chamberlain line were noted. BI is diagnosed once the tip of the odontoid is 5 mm or more above this line. <h3>DISCUSSION/CONCLUSIONS</h3> The diagnosis of BI relies on radiographic evaluation and various radiographic reference lines such as the Chamberlain line, McGregor line, or McRae line. However, the radiographic interpretation of the cervical vertebrae is often challenging and results in missing important findings. This case report aimed to demonstrate the characteristic radiographic features of BI as seen on CBCT and the use of craniometric measurements to assist health care providers in diagnosing and managing this condition. It is important for oral and maxillofacial radiologists to pay attention to this anatomically critical junction on every study that captures it and perform an assessment so they do not miss BI-related anomalies in asymptomatic patients.

Cranio-Vertebral Junction Triangular Area: Quantification of Brain Stem Compression by Magnetic Resonance Images.

(1) Background: Most of the currently used radiological criteria for craniovertebral junction (CVJ) were developed prior to the popularity of magnetic resonance images (MRIs). This study aimed to evaluate the efficacy of a novel triangular area (TA) calculated on MRIs for pathologies at the CVJ. (2) Methods: A total of 702 consecutive patients were enrolled, grouped into three: (a) Those with pathologies at the CVJ (n = 129); (b) those with underlying rheumatoid arthritis (RA) but no CVJ abnormalities (n = 279); and (3) normal (control; n = 294). TA was defined on T2-weighted MRIs by three points: The lowest point of the clivus, the posterior-inferior point of C2, and the most dorsal indentation point at the ventral brain stem. Receiver operating characteristic (ROC) analysis was used to correlate the prognostic value of the TA with myelopathy. Pre- and post-operative TA values were compared for validation. (c) Results: The CVJ-pathology group had the largest mean TA (1.58 ± 0.47 cm2), compared to the RA and control groups (0.96 ± 0.31 and 1.05 ± 0.26, respectively). The ROC analysis calculated the cutoff-point for myelopathy as 1.36 cm2 with the area under the curve at 0.93. Of the 81 surgical patients, the TA was reduced (1.21 ± 0.37 cm2) at two-years post-operation compared to that at pre-operation (1.67 ± 0.51 cm2). Moreover, intra-operative complete reduction of the abnormalities could further decrease the TA to 1.03 ± 0.39 cm2. (4) Conclusions: The TA, a valid measurement to quantify compression at the CVJ and evaluate the efficacy of surgery, averaged 1.05 cm2 in normal patients, and 1.36 cm2 could be a cutoff-point for myelopathy and of clinical significance.

Endoscopic Endonasal Transclival Odontoidectomy for Basilar Invagination: Operative Video and Technical Nuances.

Basilar invagination is a congenital or acquired craniovertebral junction abnormality where the tip of the odontoid process projects through the foramen magnum which can cause severe symptomatic compression of the brainstem and spinal cord. If left untreated, patients can develop progressive quadriparesis. Traditionally, basilar invagination can be treated with cervical traction and posterior stabilization. However, in irreducible cases, anterior decompression via a transoral or endonasal approach may be necessary. In this operative video, we demonstrate an endoscopic endonasal transclival approach for odontoidectomy to successfully treat a 37-year-old female with severe basilar invagination causing symptomatic compression on the cervicomedullary junction resulting in unsteady gait and motor weakness. The patient had Klippel–Feil syndrome where the C1 arch was assimilated to the foramen magnum and transclival drilling was needed to adequately access the odontoid process for removal. A second-stage posterior occipitocervical stabilization and fusion was performed the following day. Immediate postoperative imaging showed excellent decompression of the cervicomedullary junction. Postoperatively, the patient had significant improvement in gait and motor strength in all extremities, and was ambulating independently without assistance at 1 year after surgery. The endoscopic endonasal transclival odontoidectomy is a useful strategy to treat severe irreducible basilar invagination causing symptomatic neural compression. The surgical technique and nuances are described in a step-by-step fashion in this illustrative operative video.The link to the video can be found at: https://youtu.be/HL4K7KqJEJM.

Embryological considerations and evaluation of congenital anomalies of craniovertebral junction: A single-center experience

Objectives:Craniovertebral junction (CVJ) abnormalities constitute a group of treatable neurological disorders, especially in the Indian subcontinent. Thus, it is essential that clinicians should be able to make a precise diagnosis of abnormalities and rule out important mimickers on multidetector computed tomography (MDCT) as this information ultimately helps determine the management, prognosis, and quality of life of patients. CVJ is the most complex part of the cervical region. Congenital malformations of this region can cause serious neurological deficit and require a surgical intervention. The present study was undertaken to know the embryological basis of the CVJ and to identify commonly observed congenital CVJ abnormalities, their frequency, and mode of presentation.Materials and Methods:Diagnosed cases of CVJ anomalies on dynamic MDCT head were reviewed at a tertiary care center between January 2014 to December 2019. Type of anomaly, clinical presentation, and associated malformations were recorded. Different types of variations were expressed in terms of percentage.Results:Congenital anomalies were seen in 42 cases. Fifteen types of anomalies were detected. Anomalies were either singly or in combination. The CVJ anomalies were more common in young adults (28%), almost equal in both sexes. The most common anomaly was basilar invagination (52.3%), followed by atlanto-occipital assimilation (33.3%), and Arnold–Chiari malformation is the most common soft tissue anomaly. In fourteen cases, additional anomalies of other vertebrae were present. The most common symptoms were weakness of extremities, neck pain, paresthesia, torticollis, and gait disturbances. About 28 patients got improved, 8 patients had residual deficit as that of preoperative status, and 4 patients got deteriorated after surgery, at 1-month follow-up. About 34 patients had improved, 5 remained static, and 3 patients got worsened at the end of 3-month follow-up. About 37 patients had improved, 4 patients remained static, and 2 patients got deteriorated at 6 months of follow-up. The patients with increased atlantodens interval 3–5 mm showed 77% improvement after surgery.Conclusion:Congenital CVJ anomalies, though rare, are fatal. CVJ abnormalities constitute an important group of treatable neurological disorders with diagnostic dilemma. The atlantodental interval is the most important preoperative prognostic marker. Dynamic CT imaging can provide additional useful information to the diagnosis of CVJ instability. To prevent long-term neurological problems, early diagnosis and treatment of congenital bony CVJ anomalies is important.

Association between craniovertebral junction abnormalities and syringomyelia in patients with chiari malformation type-1.

Objectives:To assess the correlation between craniovertebral junction (CVJ) abnormalities and syringomyelia in patients with Chiari malformation type-1 (CM1).Methods:This was a retrospective study including patients with CM1. Identification of cases was done by searching a radiology database at a university hospital from 2012 to 2017. Patients were divided into 2 groups based on whether CVJ abnormalities were present (CVJ+) or absent (CVJ-). The patients’ demographic and clinical data were reviewed. All magnetic resonance imaging studies were examined by a certified neuroradiologist.Results:Sixty-four consecutive patients with CM1 were included. The mean age was 24±17 years; 59% were females. The CVJ+ group had more female patients (p = 0.012). The most frequent CVJ abnormality was platybasia (71%), followed by short clivus (44%) and cervical kyphosis (33%). The CVJ abnormalities were more in Syringomyelia cases (p = 0.045). However, the results were not significant when hydrocephalus cases were excluded.Conclusion:Among CM1 patients, CVJ abnormalities were found more in patients with syringomyelia. Future studies with larger sample size are required to further study the correlation between CVJ abnormalities and both syringomyelia and hydrocephalus in CM1 patients.

Occipito -cervical fusion in Cranio-Vertebral anomalies with basilar invagination

Basilar invagination are uncommon syndrome that occurs when the superior part of the odontoid (part of the C2 vertebrae) migrates upward through foramen magnum and that causes bulbomedullary compression. Basilar invagination are divided on bases of that causation in trau matic and other one is developmental. Mostly seen basilar invagination are developmental. Here with describe a case of 13yr old female with developmental craniovertebral junction abnormality in form of congenital atlanto axial dislocation with Platiebasia with basilar invagination which is treated with decompression and occipital plate and screw based posterior fixation with its clinical as well as radiological outcome. Keywords: Basilar invagination, AtlantoAxial dislocation, Platybasia, OC Fusion, Craniovertebral junction anomaly, Brainstem compression.

Three-Dimensional-Printed Model-Assisted Management of Craniovertebral Junction Abnormalities: An Institutional Experience with Literature Review.

Study DesignProspective study.PurposeTo evaluate the utility and limitations of using three-dimensional (3D)-printed models for the management of craniovertebral (CV) junction abnormalities.Overview of LiteratureIn comparison to other bony and vascular anomalies, CV junction abnormalities are difficult to treat. For cases of irreducible atlantoaxial dislocation (AAD), posterior reduction and stabilization have replaced anterior decompression as the standard management protocol. The use of 3D models, such as those described herein, can provide surgeons with in-depth knowledge of the vertebral artery course and bony anomalies associated with CV junction abnormalities.MethodsClinical and radiological features of 18 patients with CV junction abnormalities were analyzed between March 2017 and February 2019 at Sawai Man Singh Medical College, Jaipur, India. Dynamic computed tomography (CT) of the CV junction and CT angiographies of the neck with respect to the vertebral artery course at the C1–C2 joints were obtained and studied. Customized 3D models of the CV junction were then made based on the CT data, and rehearsal of the surgical procedure was performed using the 3D model one day prior to performing the actual procedure.ResultsSeventeen patients had congenital-type AAD, whereas one patient had posttraumatic AAD. Improvements in neck pain and myelopathy were seen in all patients at the follow-up, as analyzed using the Visual Analog Scale and the Japanese Orthopedic Association Scale score, respectively. There were no cases of malpositioning of screws or any direct vertebral artery injury, although in one patient, the distal flow in the dominant vertebral artery was cut off as it got compressed between the bony arch and the screw head.ConclusionsCompared to computer-generated images, 3D-printed models are a more practical approach for dealing with complex CV junction abnormalities. They provide surgeons with deep insights into the complex bony anomalies as well as variations in the vertebral artery courses, thereby improving surgical outcomes.

Evaluation of congenital anomalies of craniovertebral junction by computed tomography and its embryological basis

Introduction: Craniovertebral junction (CVJ) is the most complex part of the cervical region. Congenital malformations of this region can cause serious neurological deficit and require a surgical intervention. The present study was undertaken to know the embryological basis of the CVJ and to identify commonly observed congenital CVJ abnormalities, their frequency and mode of presentation. Materials and Methods: Diagnosed cases of CVJ anomalies on computed tomography (CT) head were reviewed. Type of anomaly, clinical presentation, other associated malformations were recorded. Different types of variations were expressed in terms of percentage. Result: Congenital anomalies were seen in 26 cases. Fifteen types of anomalies were detected. Anomalies were either singly or in combination. Most common anomaly was basilar invagination (23.8%) followed by atlanto-occipital assimilation (19%). In seven cases additional anomalies of other vertebrae were present. Maximum eight cases were detected in age group of 11-20 years. There was male predominance. Commonest symptoms were weakness of extremities, neck pain, paresthesia, torticollis and gait disturbances. Conclusion: Congenital CVJ anomalies are rare. To prevent long-term neurological problems, early diagnosis and treatment of congenital bony CVJ anomalies is important. Keywords: Craniovertebral junction, Congenital, Anomaly.

A Dual Approach for the Management of Complex Craniovertebral Junction Abnormalities: Endoscopic Endonasal Odontoidectomy and Posterior Decompression with Fusion

BackgroundVentral brainstem compression secondary to complex craniovertebral junction abnormality is an infrequent cause of neurologic deterioration in pediatric patients. However, in cases of symptomatic, irreducible ventral compression, 360° decompression of the brainstem supported by posterior stabilization may provide the best opportunity for improvement in symptoms. More recently, the endoscopic endonasal corridor has been proposed as an alternative method of odontoidectomy associated with less morbidity. We report the largest single case series of pediatric patients using this dual-intervention surgical technique. The purpose of this study was to evaluate the surgical outcomes of pediatric patients who underwent posterior occipitocervical decompression and instrumentation followed by endoscopic endonasal odontoidectomy performed to relieve neurologic impingement involving the ventral brainstem and craniocervical junction.MethodsBetween January 2011 and February 2017, 7 patients underwent posterior instrumented fusion followed by endonasal endoscopic odontoidectomy at our unit. Standardized clinical and radiological parameters were assessed before and after surgery. A univariate analysis was performed to assess clinical and radiologic improvement after surgery.ResultsA total of 14 operations were performed on 7 pediatric patients. One patient had Ehlers-Danlos syndrome, 1 patient had a Chiari 1 malformation, and the remaining 5 patients had Chiari 1.5 malformations. Average extubation day was postoperative day 0.9. Average day of initiation of postoperative feeds was postoperative day 1.0.ConclusionsThe combined endoscopic endonasal odontoidectomy and posterior decompression and fusion for complex craniovertebral compression is a safe and effective procedure that appears to be well tolerated in the pediatric population.

Demonstration of craniocervical junction abnormalities for diagnosis of atlanto-occipital assimilation using MRI

Objectives: Atlanto-occipital assimilation (AOA) is one of the most common skeletal anomalies of the craniovertebral junction (CVJ). Because its clinical symptomatology is non-specific and it has several variations, many cases go unnoticed which may lead to additional and unnecessary radiological examinations. In this study, we aimed to present CVJ abnormalities with MRI to improve diagnostic accuracy of AOA. Methods: Cervical MRIs of the patients registered in PACS between January 2008 and October 2011 were scanned and AOA was detected in 40 cases. Sagittal FSE T1 and T2-weighted cervical MRIs and axial T2*-GRE sequence images were re-evaluated for AOA typing, anterior atlantodental interval (AADI), posterior atlantodental interval (PADI) measurements, spine fusion anomalies, basilar invagination, tonsillar herniation, myelomalacia, suboccipital muscles and vertebral arteries (VAs). Results: CVJ abnormalities were present in all cases and the most frequent association was observed in suboccipital muscles (100%) and VAs (95%). 60% of the cases had decreased PADI, 32% C2–3 vertebrae fusion, 25% increased AADI, 22.5% basilar invagination, 15% myelomalacia and 5% tonsillar herniation. Conclusion: Suboccipital muscle abnormality was found in all AOA cases whatever the severity and type of the bony fusion. VA anomaly was observed as the second most common abnormality and accompanied preferably the cases with lateral body involvement. Being aware of additional CVJ abnormalities in MRI examinations may reduce unnecessary radiological examinations by increasing the AOA diagnosis rate.

Torticollis Caused by Nontraumatic Craniovertebral Junction Abnormalities.

Torticollis could be the only symptom and sign of craniovertebral junction (CVJ) abnormality. It could be difficult to identify CVJ abnormality as a cause of torticollis due to their rarity, especially for the subjects with torticollis caused by nontraumatic CVJ abnormalities. There has been no report to focus on nontraumatic CVJ abnormalities as a cause of torticollis. The objective of this study was to report 27 patients of torticollis caused by nontraumatic CVJ abnormalities, with the aim of helping clinicians to identify nontraumatic CVJ abnormalities as a cause of torticollis. This is a retrospective cohort study including 27 subjects who had torticollis caused by nontraumatic CVJ abnormalities. The CVJ was examined in terms of atlanto-occipital angle, atlanto-axial angle, and lateral and anterior atlanto-dens intervals for the evaluation of occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral and anterior shift of the dens, respectively. Abnormalities of the lower cervical or thoracic spine were also evaluated. Occipital condylar hypoplasia, rotation of atlanto-axial joint, and lateral shift of the dens were the most common CVJ abnormalities. The 18.5% of the subjects had concurrent anomalies of lower cervical or thoracic vertebrae along with CVJ abnormalities. Each subject had 2.22 ± 1.10 types of CVJ abnormalities on average. In conclusion, comprehensive evaluation of CVJ abnormalities is recommended for differential diagnosis of subjects with unexplained torticollis. Once CVJ abnormalities are identified, concurrent abnormalities of other vertebrae need to be evaluated.