Dislocation of atlanto-axial joint with fracture odontoid process in ankylosing-spondylitis.
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Changes in the cervical spine in juvenile rheumatoid arthritis can be shown by A.P., lateral and functional views with maximal flexion and extension, and particularly by tomography. The latter is essential for proper evaluation of the atlanto-occipital and atlanto-axial joints and of the odontoid. Atlanto-axial subluxation was found in eight out of eighty-four patients. Destruction of the odontoid was found in twelve out of fifty-three patients examined by tomography. The earliest tomographic changes at the atlanto-occipital joint consists of isolated joint narrowing, erosions and fusion and were observed in sixty-four out of sixty-six patients examined. Similar findings at the atlanto-axial joint were observed in thirty-seven out of sixty-six cases. The severity of the changes correlated with seropositivity and duration of the disease. The most marked changes were found with a prolonged history and a positive Rose-Waaler reaction.
Atlanto-axial rotatory fixation is a rare but important cause of persistent torticollis. The diagnosis is confirmed by careful radiological examination of the atlanto-axial joint, also employing tomography if necessary. Treatment is skull traction in an attempt to improve the position followed by posterior atlanto-axial fusion to prevent further, and potentially disastrous, displacement. Atlanto-axial dislocation or subluxation should be looked for in all cases of acute torticollis, when reduction by traction will most likely be successful and prevent the development of atlanto-axial rotatory fixation and the associated persistent torticollis.
An 18-month-old child was rendered tetraplegic below C6 in a road traffic accident. There was an associated fracture-dislocation of the atlanto-axial joint. Early management, and the probable role of autonomic dysreflexia in increasing the neurological deficit are considered.
Eighty-five children with Down syndrome, between sixteen months and eighteen years old, were evaluated for instability of the cervical spine at the atlanto-axial joint. The mean atlas-odontoid process interval was three millimeters in flexion and two millimeters in extension. Ten patients (12 per cent) exhibited abnormal intervals (4.5 millimeters or more) during either flexion or extension. The configuration of the odontoid process was considered normal in eighty patients and abnormal in another five patients (6 per cent). The correlation between the thickness of the interval and the degree of ligament laxity was statistically significant, as was the correlation between ligament laxity and age. Of the ten patients with an increased atlas-odontoid process interval, neurological deficit (hyperreflexia and clonus) developed in only one after a one-year follow-up.
Fifteen patients with atlanto-axial instability (secondary to os odontoideum in three, nonunion of an odontoid fracture in seven, acute odontoid fracture in three, and rheumatoid arthirtis in two) were treated by wedge compression arthrodesis of the atlanto-axial joint. One patient died at home eight weeks after fusion with the cause of death never established. Of the two patients with rheumatoid arthritis (ankylosing spondylitis), one had a non-union and in the other the posterior arch of the atlas fractured and the fusion had to be extended up to the occiput and down to the third cervical vertebra. The procedure is rarely indicated in patients with long-standing rheumatoid arthritis or severe osteopenia.
BACKGROUND: Although C2 pedicle screws are considered the gold standard for atlantoaxial fixation, the optimal fixation strategy for patients with high-riding vertebral arteries (HRVA) or narrow C2 pedicles (NC2P) remains controversial because of the increased risk of vertebral artery injury and the limitations of alternative fixation techniques. OBJECTIVE: To evaluate the safety, stability, and clinical efficacy of an individualized C2 screw fixation strategy incorporating vertebral artery mobilization for complex upper cervical anatomy. METHODS: A retrospective study was conducted in 312 patients who underwent C2 fixation between 2017 and 2025. Patients were categorized according to fusion method, screw laterality, and VA transposition requirement. Bone fusion rates and screw accuracy (Gertzbein-Robbins grading) were compared across groups using χ2, Fisher's exact, and multivariate logistic regression analyses to control confounders. RESULTS: All procedures were successfully completed without permanent neurovascular injury. At 6 months, the fusion rate with an atlantoaxial fusion cage was significantly higher than with interlaminar bone grafting (92.3% vs. 51.0%, p < 0.001). Unilateral C2 pedicle screw fixation combined with a contralateral alternative screw achieved comparable stability to bilateral fixation (p > 0.05). Screw placement accuracy was 100% clinically acceptable in normal anatomy and 60% in cases requiring VA mobilization, with no VA injury or blood flow compromise. CONCLUSION: The proposed multi-strategy C2 screw placement protocol-integrating fusion cage support and VA mobilization-achieves superior fusion, reliable fixation, and high safety, even in anatomically challenging conditions. This approach provides a reproducible and versatile solution for C2 instrumentation in complex craniovertebral junction surgery.
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Juvenile rheumatoid arthritis or, more correctly, juvenile chronic polyarthritis with its many clinical manifestations can be separated into the Still-syndrome with acute beginning, high fever and a high percentage of extra-articulalar, i.e. visceral symptoms, and the chronic polyarthritis in the more strict sense with non-visceral symptoms. The subsepsis allergica should be regarded as a subseptic first stage of the Still syndrome. The Still-syndrome implies a systemic disease mainly of the reticulo-endothelial system, with carditis, nephropathy, recurrent erythemas, and a progressing polyarthritis. Later symptoms are amyloidosis, chronic nephritis, myo- and pericarditis, and artheriitis necroticans. Predominanly the involvement of the kidneys is the reasons for the high mortality rate of 13%. Chronic polyarthritis in the strict sense is similar in children and adults, though in children rheumatic factors are rarely detected. The exsudative form of arthritis tends to cause early deterioration. Joint symptoms are distributed asymmetrically and show locally inflammed growth otherwise less common in Still-syndrome. Spondylitis cervicalis rapidly causes ankylosis. Atlanto-axial-arthritis with consequent atlanto-axial dislocation can be the reason for neurological disturbances. Juvenile mono- or oligo-arthritis often turns into polyarthritis; but for joints the prognosis is more favourable. In contrast, rheumatoid iridocyclitis as found in 22% of the cases causes unfavourable complications because symptoms are not noticed in time so that treatment is often too late. Juvenile spondylitis ankylosans begins with a peripheral arthritic stage which is not easily distinguished from chronic polyarthritis. The male sex, mono- or oligoarthritis of the outer extremities, pain in the heel, atlanto-axial-arthritis, iridocyclitis, and a positive HLA of 27 give a diagnostic clue. -- Characteristics of the therapy will be discussed.
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A case of atlanto-axial dislocation secondary to the presence of an "Os Odontoideum" is reported in which there was no history or evidence of trauma before the onset of symptoms. The treatment of this unusual clinical condition is discussed.
A review of 5 cases in the literature and our 2 additional cases of atlanto-axial sublux-ation in Down's Syndrome with associated neurologic deterioration suggests that posterior stabilization and fusion should be carried out initially. Posterior decompression alone, with excision of the posterior arch of C-1, may further increase atlantoaxial instability and contribute to neurologic deterioration. Lateral radiographs in flexion and extension and contrast studies are recommended to assess the reducibility of the lesion and the site of neural impingement. It is further recommended that if significant neurologic deterioration persists or worsens, an oral transpharyngeal odontoidectomy should be considered for cord decompression.
Pathological features are described, and clinico-pathological correlations are made, in 11 patients presenting the unusual combination of chronic compressive myelopathy of the upper cervical cord due to congenital atlanto-axial dislocation, and sudden haematomyelia and respiratory arrest developing at open surgery for release of pressure on the medullospinal tissues, or as a postoperative catstrophe. Necropsy and histological examination confirmed that the neuropathological substrate of intermittent or progressive quadriplegia and sensory changes (mainly deep sensory loss) was degeneration of the lateral and posterior columns. Loss of anterior horn cells of the lower cervical and upper dorsal cord led to weakness of hand and arm muscles. Acute paracentral haemorrhages occurring at the C1 to C2 or medullospinal level, in the territory of distribution of the sulcal branches of the spinal artery (due to sudden release of pressure at operation) were responsible for postoperative paralysis and sensory loss below C2 segment. In terms of the bony anomaly (dissected at necropsy), the cases fell into 2 sibgroups: a) The majority with the odontoid attached to the body of the axis, but short and ill developed, at times accompanied by other bony anomalies and a poorly formed transverse ligament. This tended to produce a fixed type of dislocation with a higher level (medullospinal) and more severe compression of the CNS. b) Fewer cases with a well formed but detached odontoid, producing a less severe myelopathy at a lower level (C1-C2), and generally more readily reducible on extension.
The radiological features of cervical spine involvement in rheumatoid arthritis are described. These include atlanto-axial subluxation, in both the horizontal and vertical planes, sub-axial dislocation, erosions, and vertebral end plate sclerosis. Detailed radiological investigation, using inclined plane radiography, auto-tomography, tomography, and myelography may all be required to demonstrate the abnormalities. Disorders of mobility can only be assessed with dynamic views and fluoroscopy and the mechanics of these derangements vary depending on the type of subluxation encountered. The cumulative effects of the lesions conform to certain characteristic patterns and include marked reduction of neck height, disorders of mobility, and changes in the capacity of the spinal canal. The discrepancy between these changes and the clinical and neurological findings is discussed.
A modification of common methods is described for fixation of anterior and inferior atlanto-axial dislocation (AAD and IAD). In AAD a wire is passed around the atlas arch and knotted around the spinous process of the axis in a way that a figure 8 arrangement is achieved as seen in the lateral view. In IAD a wire is passed through holes in the occiput and knotted around the spinous process of the axis. A rectangular bone graft is inserted between the occiput and the spinous process of the axis, thus preventing further inferior dislocation. In both methods methyl methacrylate and bone grafts are as a rule added for further long-time stabilization. The methods have been used in 15 cases of AAD and in three cases of IAD. On radiologic examination with attention focused on the immediate postoperative fixation the follow-up time has been three months. The long-time results of fixation have been recorded with a follow-up time of six years. The fixation proved to be satisfactory in 14 patients with AAD and in the three patients with IAD. The clinical results were good in the 14 patients with AAD and in the three with IAD.
A case of atlanto-axial subluxation in a 17 year old boy with rheumatic disease is reported. The subluxation could not be reduced by cervical traction. Posterior fixation with acrilic and wire was unable to prevent the appearance of signs of spinal cord compression. The odontoid process was removed through a transoral approach leading to neurologic recovery. Treatment of atlanto-axial subluxation is discussed, emphasis being given to the indications for anterior descompression.