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Background: The literature seems fractured for the management of craniovertebral junction (CVJ)-tuberculosis (TB). Presently, non-surgical management has been in vogue for neurologically intact patients. On the contrary, severely disabled cases of CVJ-TB continue to attract discussion, tilted towards surgical intervention. We present our experience with the non-surgical management of CVJ-TB tailored to their neurological status., Methods: Authors managed 37 cases (2004-2019; age 1-57 years, mean 36 years) of CVJ-TB, of which eighteen (18/37, 48.6%) were severely disabled (Nurick grade ≥ 3) with a mean follow-up of 84 months (48-192 months). Irrespective of the clinical status and radiological findings, all patients were managed on medical management only. Needle aspiration established pathology in 23 (62.2%) cases, while 9 (24.3%) cases required drainage of an abscess. All patients received 18 months of anti-tubercular therapy (ATT). In patients with Nurick grade ≥ 3 and documented AAD, we applied halo vest for 12 months to achieve cervical immobilization. Only hard cervical collar for 3 months was prescribed in patients with no documented AAD., Results: All minimally disabled cases (Nurick grade ≤ 2, n = 19) responded favorably (n = 18) to ATT, except for an infant, who succumbed to irreversible hypoxic brain damage due to the obstructed aero-digestive gateway. Of the severely disabled cases (Nurick grade ≥ 3, n = 18), 16 cases had favorable outcomes with only external orthosis (12) and 18-month ATT. One patient succumbed to multiple cerebral infarcts, while one required realignment surgery at CVJ due to fusion in malaligned position., Conclusion: The authors conclude that the disability grading of CVJ-TB is pertinent only for assessing the functional disability of patients at presentation, with minimal relevance in deciding its management strategy. Irrespective of neurological disability, almost all patients respond favorably to external immobilization and ATT.

Introduction: Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability., Methods: The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature., Conclusion: The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.

Background: The C1 lateral mass and C2 isthmic stabilization, as introduced by Goel and Laheri and by Harms and Melcher, is a well-known fixation technique. We present the clinical and radiographic results with freehand fluoroscopy guided C1 lateral mass and C2 isthmic fixation in a consecutive series of 28 patients, evaluating the accuracy of screw placement., Methods: Twenty-eight consecutive patients suffering from post-traumatic and other C1-C2 instability were operated on between 2001 and 2010. Indications for surgery were: trauma (n = 21 cases), os odontoideum (n = 1), cranio-verterbal malformation (n = 1), and arthritis (n = 3) and idiopathic instability (n = 2). C1 lateral mass and C2 isthmic screws were placed according to the usual anatomical landmarks with lateral fluoroscopy guidance. All patients underwent a postoperative CT scan. The extent of cortical lateral or medial breach was determined and classified as follows: no breach (grade A), 0-2 mm (grade B), 2-4 mm (grade C), 4-6 mm (grade D), more than 6 mm (grade E). Grade A and B screws were considered well positioned., Results: A total of 56 C1 lateral mass and 55 C2 isthmic screws were placed. Accuracy of screw placement was as follows: 107 grade A (96.4%), four grade B (3.6%), and no grade C, D or E. Clinical and radiological follow-up showed improvement in symptoms (mainly pain) and stability of the implants at the end of the follow-up., Conclusions: Freehand fluoroscopy-guided insertion of C1 lateral mass and C2 isthmic screws can be safely and effectively performed.

We describe the multidisciplinary findings in a pre-Columbian mummy head from Southern Peru (Cahuachi, Nazca civilisation, radiocarbon dating between 120 and 750 AD) of a mature male individual (40-60 years) with the first two vertebrae attached in pathological position. Accordingly, the atlanto-axial transition (C1/C2) was significantly rotated and dislocated at 38° angle associated with a bulging brownish mass that considerably reduced the spinal canal by circa 60%. Using surface microscopy, endoscopy, high-resolution multi-slice computer tomography, paleohistology and immunohistochemistry, we identified an extensive epidural hematoma of the upper cervical spinal canal-extending into the skull cavity-obviously due to a rupture of the left vertebral artery at its transition between atlas and skull base. There were no signs of fractures of the skull or vertebrae. Histological and immunohistochemical examinations clearly identified dura, brain residues and densely packed corpuscular elements that proved to represent fresh epidural hematoma. Subsequent biochemical analysis provided no evidence for pre-mortal cocaine consumption. Stable isotope analysis, however, revealed significant and repeated changes in the nutrition during his last 9 months, suggesting high mobility. Finally, the significant narrowing of the rotational atlanto-axial dislocation and the epidural hematoma probably caused compression of the spinal cord and the medulla oblongata with subsequent respiratory arrest. In conclusion, we suggest that the man died within a short period of time (probably few minutes) in an upright position with the head rotated rapidly to the right side. In paleopathologic literature, trauma to the upper cervical spine has as yet only very rarely been described, and dislocation of the vertebral bodies has not been presented.

In the current article we review the available English literature by pubmed search engine on the topic of osteochondromas and their location in the cervical spine. The focus is to investigate the location of the lesions in cervical spine and to analyze clinical presentations and symptoms of patients, the aetiology and histopathology examination of the masses. In addition we report a rare illustrative case of a 62 year-old man with an osteochondroma of the cervical spine. The mass developed at a very slow rate for many years and produced no clinical symptoms. The location of the extradural mass in the right atlanto-axial joint of C1 and C2 is extremely rare and was not been reported so far. After preoperative CT- and MRI-imaging the entire mass could be removed.

Purpose: C1-C2 instability or painful osteoarthritis are recognised indications for posterior atlanto-axial fixation. In the traditional trans-articular C1-C2 screw fixation, up to 20% of patients cannot have safe placement of bilateral screws in the event of a medially located vertebral artery and a straight screw trajectory in the sagittal plane. The more recently developed C1-C2 fixation technique with individual C1 lateral mass screws and converging C2 pars screws can be employed in case of a medially located vertebral artery and has comparable biomechanical strength. This is a prospective observational study to investigate the advantages, the safety, and the drawbacks of posterior atlanto-axial fixation with polyaxial C1 lateral mass screws and C2 pars screws., Methods: Twelve consecutive patients with C1-2 instability (n = 11) and painful osteoarthritis (n = 1) underwent a posterior atlanto-axial fixation with polyaxial C1 lateral mass screws and C2 pars screws. The average follow-up was 16 months and all patients reached the 12-month follow-up., Findings: No hardware failure occurred in any of the patients. Correct screw placement and construct stability was found in all 12 patients (100%) at 6 and 12 months after surgery. Mean neck pain on a visual analogue scale (VAS) was 2.1 at 6 months and 2.0 at 12 months. Only transient complications were observed: one patient presented with progressive intestinal herniation through the iliac crest scar; one suffered from severe pain at the posterior iliac crest for 3 months and three patients complained of annoying pain/dysaesthesia in the C2 dermatome for 3-6 months after surgery., Conclusion: This study confirms that posterior atlanto-axial fixation with polyaxial C1 lateral mass screws and C2 pars screws is a safe and effective surgical option in the treatment of atlanto-axial instability or painful osteoarthritis.

Unlabelled: Sacral agenesis is an uncommon condition characterised by total or partial absence of the sacrum. The association of this condition with craniocervical junction abnormalities is extremely rare., Case Report: We describe a 3-year-old girl who, at the age of 8 months, while being investigated for short stature, was found to have significant craniocervical instability associated with anomalies of the odontoid peg. In addition to this she had partial sacral agenesis but genetic tests showed a normal karyotype. Due to the inherent difficulty of surgical fixation and immobilisation in an infant of her age, she was managed conservatively in a soft cervical orthosis. At the age of three years, surgery for decompression and stabilisation was deemed necessary due to the onset of neurological morbidity., Conclusion: The authors describe this extremely rare association and discuss the difficulties faced while deciding the optimum surgical strategy for managing such young children with craniocervical instability.

Background: The aim of the study was to evaluate the effectiveness, pitfalls and failures of instrumentation and fusion with titanium wires and rods in 12 h patients with craniovertebral junction instability., Methods: Among nine adult patients (mean age 48.11 years) with craniovertebral junction instability, four had basilar impression, three metastatic disease, one rheumatoid arthritis and one Down's syndrome. Three children (mean age 7.33 years) with genetic (Down's syndrome, 2 cases) and metabolic (mucopolisaccarydoses type IV, i.e. Morquio Syndrome, 1 case) disease were studied as well. Each patient underwent preoperative radiological evaluation by means of X-Ray, CT scan and MRI of the craniocervical region. Occipitocervical instrumentation with a titanium U-shaped wired rod was performed in each patient. Autologous bone fusion was performed in all but the two cancer patients, in whom polymethylmetacrylate was used. Postoperatively, all the patients used an external orthosis for 3-6 months. Post-operative X-Ray, CT and MRI were performed on each patient. The Frankel clinical scale was used to asses the outcome at follow-up which ranged from 1 to 10 years. At maximum follow up, there was either clinical improvement or stabilization recorded in all but one patient. This patient with basilar impression transiently worsened from grade D to C and a spinal cord lesion was already evident before the operation on MRI examination., Interpretation: The effectiveness of surgical management of craniovertebral junction instability by instrumentation and fusion was demonstratedly in our experience. Nevertheless, the choice of the surgical technique should be made with caution when a spinal cord lesion is revealed by preoperative neuroimaging studies.

Grisel's syndrome involves the subluxation of the atlanto-axial joint from inflammatory ligamantous laxity following an infectious process in the head or neck. It is a rare disease usually affecting children, but infrequent adult cases do occur. Today, due to the widened use of antibiotics and availability of MR imaging, some cases presenting with neck pain, resulting from the infectious inflammation of C1 and C2 but without pronounced subluxation, can be a challenging problem for most neurosurgeons. Several theories have been proposed to explain the pathogenesis of inflammatory subluxation. The primary treatment of Grisel's syndrome is medical. The underlying infectious organism must be isolated and appropriate antibiotics must be prescribed. The subluxation is reduced in holter or skeletal traction. This paper reports two cases of infection-related atlanto-axial subluxation in two adults. The literature on this subject is briefly reviewed.

Background: Reducible atlanto-axial dislocation (AAD) may cause severe motor and respiratory compromise due to recurrent spinal cord and/or brain stem impingement. To the best of the authors' knowledge, this is the first study concentrating on the classification, the protocol of the surgical management and the outcome of congenital, reducible AAD., Methods: 109 patients with congenital, reducible AAD underwent posterior stabilization. Their preoperative disability was graded as: I (n=11, 10.09%) no functional disability (a history of minor trauma led to quadriparesis that subsequently improved); II (n=31, 28.44%) independent for activities of daily living with minor disability; III (n=42, 38.53%) partially dependent on others for their daily needs; and, IV (n=25, 22.93%) totally dependent. They were classified into 4 groups depending upon their association with: a normal odontoid and posterior arch of atlas (n=27); a dysplastic odontoid and normal posterior arch (n=25); an assimilated posterior arch (n=49); and, Arnold Chiari malformation type I (n=8). Nine patients with a dysplastic odontoid had a "hypermobile" AAD with an unrestricted backward and forward movement of the axis relative to the atlas in flexion as well as in extension of the neck, respectively. The surgical procedures included Brooks' (n=12) or modified Brooks' C1-2 fusion (n=39); Goel's C1-2 fusion (3); Ransford's contoured rod fusion (n=7); Jain's occipitocervical fusion (n=47); and, transoral decompression and Jain's occipitocervical fusion (n=1). There were 6 peri-operative mortalities in the series., Findings: At follow-up (ranging from 3 months to 6 years; n=86), 64 patients had shown improvement by one grade or more; 8 patients, who had a history of transient quadriparesis but were without neurological deficits at presentation, remained in grade I; 11 had achieved stabilization of neurological functions; while 3 had deteriorated despite adequate radiological reduction of AAD and fusion of the construct. A follow-up of 6 months or more was available in 79 of these 86 patients, in whom a dynamic intrathecal CT scan showed a good osseous union., Interpretation: The patients with congenital reducible AAD, depending on their surgical management, may be classified into four groups. Some patients with a dysplastic odontoid have a "hypermobile" AAD and require special care during intubation, positioning and stabilization. An assimilated posterior arch is often associated with asymmetrical lateral occipito-C1-C2 joint synostosis rendering transarticular screw placement difficult. The various causes of failure of constructs are discussed.

The authors report on the development of myelopathy in a case of hypoplastic upper cervical spine with atlanto-axial instability. Its surgical treatment with transarticular screw fixation and interlaminar fusion is outlined.