Anatomical evaluation of the craniovertebral junction on cone-beam computed tomography images.

INTRODUCTION: There are many craniometric measurements that are used in evaluating craniovertebral junction (CVJ) pathologies such as basilar invagination, atlantoaxial dislocation and platybasia. Therefore, it is important to determine the normal reference range of the craniometric measurements. This study aims to determine the morphometric reference values of the bony structures in the CVJ from cone-beam computed tomography (CBCT) images of a group of South Eastern Anatolian population. MATERIALS AND METHODS: The CBCT images of 300 individuals were retrospectively evaluated. 14 parameters on midsagittal and 2 parameters on the coronal plane were studied. The data were statistically evaluated. RESULTS: Measurement results were found as follows; the distances between odontoid process (OP) and McGregor line as 0.31 ± 3.22 mm, OP and Chamberlain line 1.06 ± 3.22 mm, OP and McRae line 5.30 ± 1.59 mm, OP and Fischgold digastric line 8.70 ± 4.12 mm, OP and Fischgold bimastoid line - 5.15 ± 4.86 mm, length of McRae line 35.58 ± 2.52 mm, atlantodental interval 1.28 ± 0.48 mm, posterior atlantodental interval 19.54 ± 2.24 mm, basion axial interval 4.01 ± 1.83 mm, basion dental interval 4.92 ± 1.77 mm, length of Modified Ranawat line 28.66 ± 2.38 mm, length of Redlund-Johnell line 35.11 ± 4.09 mm, clivus canal angle 157.62° ± 11.85°, Welcher basal angle 130.83° ± 6.29°, craniocervical tilt 126.98° ± 12.24° and Powers ratio as 0.72 ± 0.06. CONCLUSION: In this study, the morphometric values were evaluated according to age and gender in individuals who did not have any radiologic anomalies. The normal reference ranges may be useful for researchers who are researching pathology in this region. It is recommended to conduct further studies with different populations to determine the normal reference range.

Clivopalate angle: a new diagnostic method for basilar invagination at magnetic resonance imaging.

OBJECTIVES: To investigate the diagnostic value of clivopalate angle (CPA) for basilar invagination (BI) at magnetic resonance imaging (MRI). METHODS: In this retrospective case-control study, CPA, clivodens angle (CDA), and clivoaxial angle (CXA) were measured on midsagittal MR images from 112 patients with BI (22 men; mean age, 43.9 years ± 13.1 years; range, 21-79 years) and 200 control subjects (57 men; mean age, 47.1 years ± 13.3 years; range, 20-80 years). Intraclass correlation coefficient (ICC), linear regression, Mann-Whitney U test, binary logistic regression, and receiver operating characteristic (ROC) curve were used for statistical analysis. RESULTS: Clivopalate angle showed better inter-observer agreement (ICC = 0.951) than CXA (0.867) or CDA (0.853). CPA significantly correlated with CXA (R = 0.811, p < 0.001) and CDA (R = 0.716, p < 0.001). Patients with BI had a significantly smaller CPA (45.9° ± 9.9°) than control subjects (61.9° ± 6.2°) (p < 0.001). With the optimal cutoff value of 53.5°, CPA had a sensitivity of 0.839 (94/112) and a specificity of 0.915 (183/200). The area under the ROC curve (AUC) was 0.937 (95% CI, 0.911-0.963) for CPA, which was similar to that of CXA (AUC, 0.957; 95% CI, 0.936-0.978) or CDA (AUC, 0.925; 95% CI, 0.892-0.957). The combination of CPA and CDA or CXA showed a higher diagnostic value than CDA or CXA alone. CONCLUSIONS: The diagnostic performance of CPA was similar to that of CXA or CDA, but CPA might be more reliable in evaluation of BI. CPA provided complementary information to CXA and CDA. KEY POINTS: • Clivopalate angle has a high diagnostic value for basilar invagination. • Clivopalate angle demonstrates high inter-reader agreement than does clivoaxial angle or clivodens angle. • Clivopalate angle provides complementary information to clivoaxial angle and clivodens angle.

Visual and Positional Modulation of Pendular Seesaw Nystagmus: Implications for the Mechanism.

BACKGROUND: The mechanisms of pendular seesaw nystagmus (SSN) remain unknown. METHODS: We evaluated modulation of pendular SSN by removal of visual fixation, convergence, and positional changes in 2 patients, one with bitemporal hemianopia due to a traumatic damage of the optic chiasm and the other with platybasia compressing the medulla and lower cerebellum. RESULTS: In both patients, the pendular SSN markedly decreased or disappeared with convergence, without visual fixation in darkness, during static head tilt toward each shoulder while sitting and while supine. CONCLUSIONS: The similar patterns of nystagmus modulation observed in our patients with a different etiology indicate a common role of both visual and otolithic inputs in generating pendular SSN.

[The rheumatic cervical spine]. / Die rheumatische Halswirbelsäule.

BACKGROUND: The cervical spine is one of the main sites of manifestation in rheumatoid arthritis outside of the extremities. It can have a decisive influence on disease course via the occurrence of mechanical instabilities as well as neurologic symptoms. Both adequate diagnosis and the corresponding surgical treatment represent a challenge for the involved physicians. MATERIALS AND METHODS: This review presents relevant diagnostic strategies and possibilities for surgical intervention which aim to avoid potentially fatal neurologic symptoms. Basic literature and expert opinions are also discussed. RESULTS AND CONCLUSION: Through target-oriented surgical management, as well as tight clinical and radiologic monitoring during conservative and surgical therapy, potentially fatal disease courses can be avoided.

Dysphagia as a primary manifestation of basilar impression: A case report.

Secondary basilar invagination or basilar impression is an anomaly at the craniovertebral junction where the odontoid process prolapses into the foramen magnum with the risk of compressing adjacent structures and obstructing the proper flow of cerebrospinal fluid (CSF). The incidence is less than 1% in the general population and occurs mainly in the first three decades of life when it is associated with malformations of the neuroaxis. In older age, the main aetiologies are diseases that alter bone mineral density. The clinical course is usually progressive and the most common symptoms are asthenia, cervical pain and restricted movement, but also dysphonia, dyspnoea and dysphagia. It is a progressive disease which, if left untreated, can cause severe neurological damage and death. We report the case of a 79-year-old woman with osteoporosis and progressive dysphagia leading to severe malnutrition, which conditioned the decision not to intervene due to the high perioperative risk.

A novel classification and management scheme for craniocervical junction disorders with ventral neural element compression.

OBJECTIVE: Craniocervical junction (CCJ) pathologies with ventral neural element compression are poorly understood, and appropriate management requires accurate understanding, description, and a more uniform nomenclature. The aim of this study was to evaluate patients to identify anatomical clusters and better classify CCJ disorders with ventral compression and guide treatment. METHODS: A retrospective review of adult and pediatric patients with ventral CCJ compression from 2008 to 2022 at a single center was performed. The incidence of anatomical abnormalities and compressive etiologies was assessed. Surgical approach, radiographic data, and outcomes were recorded. Association rules analysis (ARA) was used to assess variable clustering. RESULTS: Among 51 patients, the main causes of compression were either purely bony (retroflexed dens [n = 18]; basilar invagination [BI; n = 13]) or soft tissue (degenerative pannus [n = 16]; inflammatory pannus [n = 2]). The primary cluster in ARA was a retroflexed dens, platybasia, and Chiari malformation (CM), and the secondary cluster was BI, C1-2 subluxation, and reducibility. These, along with degenerative pannus, formed the three major classes. In assessing the optimal treatment strategy, reducibility was evaluated. Of the BI cases, 12 of the 13 patients had anterolisthesis of C1 that was potentially reducible, compared with 2 of the 18 patients with a retroflexed dens (both with concomitant BI), and no pannus cases. The mean C1-2 facet angle was significantly higher in BI at 32.4°, compared with -2.3° in retroflexed dens and 8.1° in degenerative pannus (p < 0.05). Endonasal decompression with posterior fixation was performed in 48 (94.0%) of the 51 patients, whereas posterior reduction/fixation alone was performed in 3 patients (6.0%). Of 16 reducible cases, open posterior reduction alone was successful in 3 (60.0%) of 5 cases, with all successes containing isolated BI. Reduction was not attempted if vertebral anatomy was unfavorable (n = 9) or the C1 lateral mass was absent (n = 5). The mean follow-up was 28 months. Symptoms improved in 88.9% of patients and were stable in the remaining 11.1%. Tracheostomy and percutaneous G-tube placement occurred in 7.8% and 11.8% of patients, respectively. Reoperation for an endonasal CSF leak repair or posterior cervical wound revision both occurred in 3.9% of patients. CONCLUSIONS: In classifying, one cluster caused decreased posterior fossa volume due to an anatomical triad of retroflexed dens, platybasia, and CM. The second cluster caused pannus formation due to degenerative hypertrophy. For both, endonasal decompression with posterior fixation was ideal. The third group contained C1 anterolisthesis characterized by a steep C1-2 facet angle causing reducible BI. Posterior reduction/fixation is the first-line treatment when anatomically feasible or endonasal decompression with in situ posterior fixation when anatomical constraints exist.

Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation.

A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes. Radiographs showed occipitalization of the atlas, C2-C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ) compression from the odontoid, a Chiari type I malformation, and syringomyelia extending from the foramen magnum to C5. The patient underwent transoral atlantoaxial release followed by posterior internal fixation from the occiput to the axis, which resulted in a significant improvement in motor function in all extremities. Post-operative images showed anatomical reduction of the atlantoaxial joint. However, an MRI performed 8 days following surgery showed a new retro-odontoid pannus had developed that was compressing the spinal cord at CMJ. A follow-up CT scan performed at 6 months post-operatively demonstrated a solid bony fusion between the occiput and C2, while an MRI at that time showed complete resolution of the retro-odontoid soft tissue mass with correction of the Chiari I malformation, and resolution of the syringomyelia. Final follow-up at 2-years revealed an excellent clinical outcome.

Basilar invagination, Chiari malformation, syringomyelia: a review.

Institute and personal experience (over 25 years) of basilar invagination was reviewed. The database of the department included 3300 patients with craniovertebral junction pathology from the year 1951 till date. Patients with basilar invagination were categorized into two groups based on the presence (Group A) or absence (Group B) of clinical and radiological evidence of instability of the craniovertebral junction. Standard radiological parameters described by Chamberlain were used to assess the instability of the craniovertebral junction. The pathogenesis and clinical features in patients with Group A basilar invagination appeared to be related to mechanical instability, whereas it appeared to be secondary to embryonic dysgenesis in patients with Group B basilar invagination. Treatment by facetal distraction and direct lateral mass fixation can result in restoration of craniovertebral and cervical alignment in patients with Group A basilar invagination. Such a treatment can circumvent the need for transoral or posterior fossa decompression surgery. Foramen magnum bone decompression appears to be a rational surgical treatment for patients having Group B basilar invagination. The division of patients with basilar invagination on the basis of presence or absence of instability provides insight into the pathogenesis of the anomaly and a basis for rational surgical treatment.

Endoscopic transnasal decompression for management of basilar invagination in osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a disorder of bone development caused by a genetic dysfunction of collagen synthesis. Basilar invagination (BI) is an uncommon but serious complication of OI. Brainstem decompression in OI is undertaken in certain circumstances. Transoral-transpalatopharyngeal ventral decompression with posterior occipitocervical fusion has become the treatment of choice when required. This technical note outlines a novel endoscopic transnasal approach for ventral decompression. The literature is reviewed and a strategy for the management of BI in patients with OI is outlined.

Basilar impression and osteogenesis imperfecta: a 21-year retrospective review of outcomes in 20 patients.

OBJECT: Basilar impression (BI) secondary to osteogenesis imperfecta (OI) is a rare but debilitating condition that is often progressive unless it is halted. More recently, ventral decompression surgery has been advocated for this condition. This study is a retrospective review of the 21-year experience of ventral decompression surgery and dorsal occipitocervical fixation in patients with BI secondary to OI and is the largest patient series reported to date. METHODS: Twenty patients treated between 1982 and 2003 by the senior author at the authors' institution were included in this study. All patients underwent ventral decompression surgery followed by dorsal craniocervical stabilization. Patients were followed up for a median of 10 years. RESULTS: There were no intraoperative or perioperative deaths. Postoperatively, 16 of 20 (80%) patients showed objective improvement or maintained their good preoperative level of function. After surgery, of the 15 patients admitted with Karnofsky Performance Scale (KPS) scores of 70% or less, 11 improved, two remained unchanged, one patient's condition deteriorated, and one patient died of an unrelated cause. Of five patients admitted with a KPS score of 80% or greater, no patient's condition deteriorated in the short- and midterm period, but one patient had recurrence 15 years after surgery. At the end of follow-up, 25% of the patients had recurrence of brainstem compression symptoms or had died, and 15% showed no improvement after surgery. All of the remaining patients (60%) had sustained a long-term benefit from surgery. CONCLUSIONS: Aggressive ventral decompression surgery and dorsal stabilization for patients with BI secondary to OI can not only halt disease progression but can also produce a good and sustainable long-term functional outcome, even in those patients who present as severely symptomatic. Patients who presented early with minor symptoms had good long-term outcomes.

Endoscopic image-guided odontoidectomy for decompression of basilar invagination via a standard anterior cervical approach. Technical note.

Symptomatic irreducible basilar invagination has traditionally been approached through a transoral-transpharyngeal route with resection of the anterior portion of C-1 and the odontoid. Modification of this exposure with either a Le Fort osteotomy or a transmandibular osteotomy and circumglossal approach has increased the access to pathological conditions in this region. These traditional routes all require traversing the oral cavity and accepting the associated potential complications. The authors have developed a novel surgical approach, an endoscopic transcervical odontoidectomy, which allows access for resection of the odontoid and for brainstem and spinal cord decompression without traversing the oral cavity. In this paper they describe the technique and its advantages and present three cases in which patients underwent the endoscopic transcervical odontoidectomy for basilar invagination. Three consecutive patients (age range 42-74 years) who had irreducible basilar invagination underwent the endoscopic transcervical odontoidectomy. All were symptomatic and had neck pain and myelopathy. All were evaluated preoperatively and postoperatively with computed tomography and magnetic resonance imaging. In all cases the procedure resulted in complete decompression. There were no serious complications. No patient required prolonged intubation, tracheostomy, or enteral tube feeding. One patient had an intraoperative cerebrospinal fluid leak, which had no postoperative sequelae. The authors present an alternative surgical approach for treating ventral compression of the brainstem and spinal cord. The technique is safe and effective for decompression and provides a surgical route that can be added to the armamentarium of treatments for pathological conditions in this region.

Neuronavigation-assisted transoral-transpharyngeal approach for basilar invagination--two case reports.

Two patients presented with congenital basilar invagination manifesting as progressive myelopathy. Both patients underwent surgery using a neuronavigation-assisted transoral-transpharyngeal approach. The Brain-LAB Vector Vision navigation system was used for image guidance. The registration accuracies were 0.9 and 1.3 mm. After decompression, posterior stabilization was performed. Both patients had an uneventful postoperative course. The transoral-transpharyngeal approach with the neuronavigation system provides safe exposure and decompression for basilar invagination.

Basilar impression associated with impacted cisterna magna, spastic paraparesis and distress of balance: case report.

We report on a 48 years-old man with basilar impression without syringohydromyelia, in which the cisterna magna was impacted by the cerebellar tonsils. Six months after posterior fossa decompression there was the disappearance of nuchal rigidity, vertigo, spastic paraparesis and improvement of balance. Nevertheless hyperreflexia and diminished pallesthesia of the lower limbs persisted.

Evaluation of platybasia with MR imaging.

BACKGROUND AND PURPOSE: Platybasia, or abnormal obtuseness of the basal angle, was first measured on plain skull images. At present, evaluation of the brain and skull more commonly involves CT and MR imaging. We evaluated a new MR imaging method of evaluating platybasia. METHODS: We retrospectively evaluated midline sagittal MR images in 200 adults and 50 children. The basal angle of the skull base was measured by using two methods: The standard MR imaging technique measured the angle formed by two lines-one joining the nasion and the center of the pituitary fossa connected by a line joining the anterior border of the foramen magnum and center of the pituitary fossa. The modified technique measured the angle formed by a line across the anterior cranial fossa and dorsum sellae connecting a line along the clivus. RESULTS: With the standard MR imaging technique, we obtained mean angles of 129 degrees +/- 6 degrees for adults and 127 degrees +/- 5 degrees for children, compared with 135.3 degrees (composite mean) in previous series. The modified technique produced values of 117 degrees +/- 6 degrees for adults and 114 degrees +/- 5 degrees for children, which were significantly lower that those of standard MR imaging and traditional radiography (P <.05). CONCLUSION: Both the standard and modified MR imaging techniques produced basal angles lower than those previously reported with standard radiography. The modified technique uses clearly featured landmarks that can be reproduced consistently on midline sagittal T1 images. This technique and its corresponding values can be used as the new standard for evaluating the basal angle.

Magnetic resonance imaging study of the craniocervical junction.

PURPOSE: To confirm the accuracy of the standard parameters of Chamberlain's line, McGregor's line, McRae's line, and Ranawat's line in the diagnosis of basilar impression in a normal Asian population using magnetic resonance imaging (MRI) scans of the craniocervical junction, and to evaluate the use of MRI in the initial diagnosis of basilar impression. METHODS: We retrospectively reviewed MRI scans of the craniocervical junction in 114 Thai patients (61 men and 53 women) aged 20 to 89 years to assess the lines of Chamberlain, McGregor, McRae, and Ranawat. Patients with injury, tumour, or metastasis in the craniocervial junction; congenital anomalies; or clinical symptoms and signs of basilar impression were excluded. RESULTS: 39 of the 114 patients had the odontoid tip extended above Chamberlain's line by 1 mm to 9 mm (mean, 2.89 mm). Only 7 men and 5 women had the tip of the odontoid process extended beyond the normal range of 5 mm. The mean distance of the odontoid tip extended past McGregor's line in male and female subjects was 0.33 mm (standard deviation [SD], 1.2 mm) and 0.17 mm (SD, 0.7 mm), respectively, markedly less than those reported previously, although one male patient's odontoid tip extended 8 mm above the McGregor's line. Regarding McRae's line, no patient had the odontoid tip extended above the foremen magnum. The mean Ranawat's line in male and female subjects was 15.75 mm (range, 12-19; SD, 1.71 mm) and 14.09 mm (range, 11-17; SD, 1.38 mm), respectively. CONCLUSION: After using MRI to confirm the standard parameters of basilar impression as previously formulated from lateral skull radiographs, we recommend using McRae's line and Ranawat's line in the initial screening for basilar impression for the Asian population. Chamberlain's line and McGregor's line are viable alternatives, but clinicians must be aware of the potential for false positives.

Craniocervical CT and MR imaging of Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome is a rare, inherited disorder characterized by myotonia, skeletal deformities, facial dysmorphism, and growth retardation. In this report of an adolescent male patient with Schwartz-Jampel syndrome, CT and MR imaging revealed basilar invagination, platybasia, Chiari I malformation, hyperpneumatized mastoids with intramastoid dural sinuses, platyspondyly, bulbous zygoma, and blunted pterygoid processes.

The operative management of basilar impression in osteogenesis imperfecta.

Four patients with osteogenesis imperfecta and neurologically significant basilar impression have been treated over the past 8 years. The experience has resulted in changes in our therapeutic strategy for this particularly difficult problem. These cases are discussed with respect to the disease process, neurological involvement, radiological findings, and modes of surgical therapy. The errors in management as well as the success resulting from our learning experience are described. Currently, we recommend the extensive removal of the anterior bony compression by a transoral approach. This should be followed by a posterior rigid fixation that transfers the weight of the head to the thoracic spine, in an effort to prevent further basilar invagination.

Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation.

Cine-mode magnetic resonance imaging provides simultaneous images of cerebrospinal fluid flow dynamics. A patient with a basilar impression accompanied by a Chiari malformation and von Recklinghausen's disease who underwent transoral decompression is reported. Preoperative cine-mode magnetic resonance imaging visualized an associated obstruction of cerebrospinal fluid pulsatile flow at the level of the foramen magnum. Tonsilar herniation (Chiari I malformation) and hydrocephalus were also present. Postoperatively, the obstruction of cerebrospinal fluid flow was resolved concomitant with the correction of the cervicomedullary angulation. On the basis of observations made by magnetic resonance imaging, the surgical treatment of basilar impression accompanied by Chiari malformation is briefly discussed.

Total craniospinal decompression in achondroplastic stenosis.

We describe our experience with total craniospinal decompression along the entire neuraxis, extending from the brain stem to the cauda equina, in seven patients with achondroplasia. These patients presented with clinically significant compression at multiple levels. In these patients, there were focal areas of complete myelographic block, typically at the cervicothoracic or thoracolumbar junction, as well as diffuse narrowing of the entire spinal subarachnoid space. In some, there were further complications of basilar impression, Arnold-Chiari malformation, or syringomyelia. Total craniospinal decompression was completed in either one or two stages. Only a small minority of our patients with achondroplasia had critical stenosis over this many levels, requiring total craniospinal decompression. However, with proper preparation and technique, we found that patients can tolerate even such an extensive decompressive procedure and benefit from surgery without suffering postoperative spinal instability.