Atlantoaxial Subluxation Research Articles

A rare case of Klippel-Feil syndrome associated with atlantoaxial rotatory subluxation, atlanto-occipital fusion, and spina bifida

A rare case of Klippel-Feil syndrome associated with atlantoaxial rotatory subluxation, atlanto-occipital fusion, and spina bifida

Grisel's syndrome with hematomyelia

Grisel's syndrome is a rare condition characterized by atlantoaxial subluxation following an inflammatory process in the head and neck region. It occurs more commonly in children and usually presents with cervical pain and torticollis, in addition to symptoms of the primary infection. A case of Grisel's syndrome is discussed here who suddenly developed quadriplegia with severe respiratory compromise and ultimately diagnosed as reducible atlantoaxial subluxation with hematomyelia. Hematomyelia was extending from medulla to the third cervical vertebrae level of cord. The case was managed successfully by posterior fusion. Though the clinical improvement occurred following posterior fusion, but there was no optimum radiological reduction of the reducible atlantoaxial subluxation. This case emphasizes the importance of posterior fusion in Grisel's syndrome where an offending posterior indentation of upper cervical cord by dislocated posterior arch, if dealt with; the clinical improvement can be achieved even without sufficient anterior reduction of subluxation. The reasons of hematomyelia and nonreduction atlantoaxial subluxation are discussed here.

Persistent torticollis: Think about Grisel's syndrome

Grisel's syndrome (GS) is defined as a non-traumatic subluxation of the atlanto-axial joint and is considered a rare complication of infectious or inflammatory processes of the head and neck region due to upper respiratory tract infections or following surgical otolaryngeal interventions. The etiopathogenesis of this clinical entity is not yet clear. Early recognition of pediatric atlanto-axial subluxation (AAS) is essential to avoid permanent damage and requires a combination of clinical assessment and appropriate radiologic imaging. Computed tomography (CT) is considered gold standard in the diagnosis of GS. We report a case of a young child who presented with torticollis and acute otitis media and discuss the potential role of magnetic resonance imaging (MRI) in first suggesting the diagnosis. A brief review of the literature is presented. Conclusion: radiologists should first consider MRI of the atlanto-axial joint in young patients with torticollis with a recent history of infection in the head and neck region.

Conservative treatment for Landells and Van Peteghem type II atlas fracture accompanied with atlantoaxial rotatory subluxation

Conservative treatment for Landells and Van Peteghem type II atlas fracture accompanied with atlantoaxial rotatory subluxation

Quantitative Analysis of Cervical Flexion-Extension Radiographs in Rheumatoid Arthritis Patients

Diagnostic retrospective case series. To evaluate cervical intersegmental motion in rheumatoid arthritis patients using specialized tracking software (QMA) and compare the findings with a previously published cohort of "normal" subjects. Rheumatoid arthritis follows 3 patterns of cervical involvement, atlantoaxial subluxation, atlantoaxial impaction, and subaxial subluxation. Deformities present are sometimes considered "unstable" to the point where surgery can be recommended based on the interpretation of cervical radiographs. Cervical flexion-extension radiographs of 99 subjects with rheumatoid arthritis were evaluated. Angular and translational segmental motions were determined at each level using specialized tracking software. Findings were compared with previously published normative data using the same device. Relative to controls, patients with rheumatoid arthritis demonstrated significantly less overall sagittal motion. Segmental analysis at C1-C2 demonstrated a 10% prevalence of dynamic motion outside the 95% confidence interval for "normal" patients. In contrast, segmental analysis at C2-C7 demonstrated no intersegmental motion outside the 95% confidence interval for "normal" subjects. In patients with rheumatoid arthritis, abnormal motion at C1-C2 was consistent with truly dynamic deformity and should be closely evaluated. Conversely, subaxial subluxations, although frequently present, were relatively fixed deformities and in most cases, may not represent an unstable condition.

Atlantoaxial rotatory subluxation in children.

Atlantoaxial rotatory subluxation is a rare condition in which patients present with the acute onset of torticollis. Atlantoaxial rotatory subluxation represents a spectrum of disease from muscle spasm to a fixed mechanical block to reduction of the atlantoaxial complex. If left untreated, some cases may resolve spontaneously; however, other cases may result in the development of secondary changes in the bony anatomy of the atlantoaxial joint, leading to persistent deformity. Diagnosis of the condition is largely clinical but can be aided by various imaging modalities, including radiographs, dynamic CT scanning, three-dimensional CT reconstructions, or MRI. Consideration should always be given to infection or other inflammatory disease as an underlying, precipitating cause. Treatments include observation, the use a cervical collar and analgesics, halter or skeletal traction, and posterior fusion of C1-C2. The most important factor for success of conservative treatment is the time from the onset of symptoms to recognition and the initiation of treatment.

Pinless halo in the pediatric population: indications and complications.

The use of the conventional halo is accompanied by the possibility of serious complications, especially in the pediatric population. Complications could include penetration of pin into the skull, pin loosening, pin tract infection, cranial nerve palsies, and vest-related pressure sores. The noninvasive "pinless" halo was introduced in an attempt to mitigate these problems while retaining the effectiveness of the conventional halo. The purpose of this study is to determine the indications and complications related to pinless halo application. We retrospectively reviewed 61 patients, whose treatment included the use of a pinless halo device, presenting to our institution between 2004 and 2012. There were 35 male and 26 female patients with an average age of 6.04 years. Indications of pinless halo application included postoperative immobilization for congenital muscular torticollis in 38 cases, conservative management of atlantoaxial rotatory subluxation in 11 cases, postoperative immobilization following cervical fusion in 10 cases, and immobilization for occipital condyle fracture in 2 cases. The average duration of the pinless halo application was 32.68 days. Thirteen patients had complications, among which major complications were seen in 2 patients, each of whom developed a pressure sore; one on the scalp and the other on the chest. Both the pressure sores responded to local treatment; however, 1 resulted in permanent alopecia. The use of the noninvasive pinless halo was found to be safe with few complications in our study. The complications were infrequent and patients were compliant to treatment, indicating that this modality is patient-friendly. Effectiveness of this treatment in comparison with invasive halos and other cervical orthoses was not determined and is a limitation of this study. Level IV-Case series.

Cervical spine involvement in rheumatoid arthritis over time: results from a meta-analysis.

IntroductionComplications in rheumatoid arthritis (RA) seem less common than they were years ago. The prevalence and progression of anterior atlantoaxial subluxations (aAASs), vertical subluxations (VSs), subaxial subluxations (SASs), and associated cervical myelopathy in RA over the past 50 years were determined.MethodsA literature search was performed by using Medline-OVID/EMBASE, PubMed, and Scopus (from 1960 to June 21, 2014). Prevalence studies were included if the sample size was at least 100 or the prevalence/progression of cervical subluxations was reported. Study quality was assessed by using the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist. Prevalence of cervical subluxations was calculated for each study. Student’s t test and meta-regression were used to evaluate for significance.ResultsIn total, 12,249 citations were identified and 59 studies were included. The prevalence of aAAS decreased from 36% (95% confidence interval (CI) 30% to 42%) before the 1980s to 24% (95% CI 13% to 36%) in the 2000s (P = 0.04). The overall prevalence rates were 11% (95% CI 10% to 19%) for VS, 13% (95% CI 12% to 20%) for SAS, and 5% (95% CI 3% to 9%) for cervical myelopathy, and there were no significant temporal changes. Rates of progression of aAAS, VS, and SAS were 4, 6, and 3 lesions per 100 patients per year, respectively. The incidence of new or progressive cervical myelopathy was 2 cases per 100 patients with known cervical subluxations per year.ConclusionsSince the 1960s, only aAAS has decreased dramatically. It is still more than twice as common as VS or SAS. No temporal changes in the development of cervical myelopathy in affected patients with RA were noted. The progression rates of cervical subluxations and myelopathy were unchanged over time.

Modification of Goel–Harms Technique in the Surgical Treatment of Atlantoaxial Subluxation after Grisel Syndrome

Introduction Many etiologies can lead to atlantoaxial subluxation. In Grisel syndrome, this subluxation occurs spontaneously after inflammatory processes of the head and neck. Nonsurgical treatment most often resolves the symptoms; however, in some cases, surgical treatment is necessary to repair the subluxation. Various surgical techniques and instrumentation systems have been used to treat atlantoaxial subluxation, although there is no consensus regarding the best treatment method for the pediatric population. We aim to describe a modified surgical technique for the treatment of atlantoaxial subluxation in a child with Grisel syndrome. Patient and Methods: Our case study involves a 5-year-old female patient with a 6-month history of unresolved Fielding type II atlantoaxial subluxation because of the Grisel syndrome. Despite conservative treatment, the patient's symptoms continued to progress. After two failed closed reduction attempts, open reduction and C1–C2 fusion with a modified Goel–Harms technique was performed with atlas laminar hook and axis pedicle polyaxial screws. A literature review of the surgical treatment of the Grisel syndrome was also performed. Results After surgery, the patient exhibited full clinical and functional recoveries with complete resolution of symptoms. At the 24-month follow-up examination, there was continual evidence of satisfactory reduction and fusion. No complications were observed. On completion of the literature review, seven Grisel syndrome cases were found to have been treated surgically with the minimum patient age being 9 years. Conclusion Conservative management of Grisel syndrome is the most common and effective treatment; however, a few surgical cases have been reported in the literature with good results. We exhibit a safe, new surgical construct for pediatric patients in the treatment of atlantoaxial subluxation and instability, with satisfactory clinical results and solid fusion at 24 months postsurgery.

Posterior Traumatic Atlantoaxial Subluxation: A Case Report

Introduction Injuries to the cervical spine are often described as neurologically catastrophic events with a very low survival rate. Lesions of the C3–C7 segment are the most common (∼ 80%) followed by the segment C1–C2 (20%). Injuries to the cervical spine are of great importance, both for its severity as well as the neurological implications they entail. The delay in diagnosis and treatment has been reported as a negative prognostic factor associated with poor outcome. Cases of atlantoaxial dislocations have been reported without neurological deficit at presentation and in long-term follow-up so these injuries could be unnoticed at the emergency department. When neurological damage is not evident at presentation symptoms are generally unspecific, such as, suboccipital pain when axial cranial pressure is applied movement, or at passive rotation the patient spontaneously assumes a stiff neck, contracting sternocleidomastoid and trapezius muscles. Materials and Methods A 28-year-old male patient without relevant medical history was a victim of physical assault on August 15, 2012, suffering cranial and cervical trauma, and was discharged, he showed no neurological symptoms then. Six weeks after discharge, he began with paresthesias of thoracic limbs and subsequently with diminished muscle strength, which was gradually decreasing. Imaging demonstrated atlantoaxial subluxation. Posterior instrumentation with transfacetary lag screws at C1/C2 was performed. Results One year after surgery, the patient presents without neurological deficit, absence of stiff neck and denies pain when mobilization of the neck or axial compression is applied. Fusion was achieved with no further complications. Conclusion The diagnosis of traumatic subluxation should be suspected in the presence of torticollis and neck pain associated with cervical trauma. However, atlantoaxial dislocation can occur in the absence of any clinical signs so it should be systematically considered as a diagnosis an should be excluded in any polytraumatized. We suggest that simple radiographic measurements (ADI and Powers Index). When the instability of the C1/C2 joint is diagnosed, early reduction, and fusion is recommended as the definitive treatment.

Reference ranges for atlantodental interval in adults and its variation with age and gender in a large series of subjects on multidetector computed tomography.

The atlanto-dental interval measurement in the cervical spine in young adults has been reported on multidetector computed tomography (MDCT), but has not been systematically assessed in all ages. To evaluate the reference ranges for the atlanto-dental interval (ADI) in a large sample of adult patients on MDCT and to analyze the relationships of these measurements with gender and age of the patients as well as inter-observer performance. First, a consecutive series of 700 Chinese patients aged over 18 years undergoing an upper cervical MDCT scan were divided equally into seven age groups. Second, the mid-sagittal image of the atlanto-dental joint was chosen for measurement and ADI was defined as the distance intercepted from the segment line linking the center of anterior atlas tubercle with that of posterior tubercle. Third, the correlation of ADI with age and gender was analyzed using linear regression analysis. Finally, the inter-observer performance was assessed using Bland and Altman's limits of agreement. The ADI was 1.77 ± 0.39 mm, 1.61 ± 0.37 mm, 1.58 ± 0.36 mm, 1.41 ± 0.29 mm, 1.31 ± 0.29 mm, 1.34 ± 0.28 mm, and 1.06 ± 0.47 mm in the age groups 18-24 years, 25-29 years, 30-39 years, 40-49 years, 50-59 years, 60-69 years, and older than 70 years, respectively. The range of ADI was 0.85-3.12 mm in the age group 18-39 years, 0.71-2.55 mm in the age group 40-59 years, and 0.00-2.37 mm in the age group older than 60 years. There was no difference between gender and ADI (r = 0.00, P = 1.000). ADI decreased linearly with age (r = -0.511, P = 0.000). The inter-observer performance was reliable. ADI measurements on MDCT linearly decrease with increasing age, and these are not affected by gender in adult patients. Different reference values might be used at different ages when diagnosing atlantoaxial anterior dislocation or subluxation.

Surgical treatment of Type II odontoid fractures: anterior odontoid screw fixation or posterior cervical instrumented fusion?

Odontoid fractures comprise as many as 20% of all cervical spine fractures. Fractures at the dens base, classified by the Anderson and D'Alonzo system as Type II injuries, are the most common pattern of all odontoid fractures and are also the most common cervical injuries in patients older than 70 years of age. Surgical treatment is recommended for patients older than 50 years with Type II odontoid fractures, as well as in patients at a high risk for nonunion. Anterior odontoid screw fixation (AOSF) and posterior cervical instrumented fusion (PCIF) are both well-accepted techniques for surgical treatment but with unique indications and contraindications as well as varied reported outcomes. In this paper, the authors review the literature about specific patients and fracture characteristics that may guide treatment toward one technique over the other. AOSF can preserve atlantoaxial motion, but requires a reduced odontoid, an intact transverse ligament, and a favorable fracture line to achieve adequate fracture compression. Additionally, older patients may have a higher rate of pseudarthrosis using this technique, as well as postoperative dysphagia. PCIF has a higher rate of fusion and is indicated in patients with severe atlantoaxial misalignment and with poor bone quality. PCIF allows direct open reduction of displaced fragments and can reduce any atlantoaxial subluxation. It is also used as a salvage procedure after failed AOSF. However, this technique results in loss of atlantoaxial motion, requires prone positioning, and demands a longer operative duration than AOSF, factors that can be a challenge in patients with severe medical conditions. Although both anterior and posterior approaches are acceptable, many clinical and radiological factors should be taken into account when choosing the best surgical approach. Surgeons must be prepared to perform both procedures to adequately treat these injuries.

One-step reduction and fixation applying transposterior arch lateral mass screw of C1 combined with pedicle screw of C2 and rod system for pediatric acute atlantoaxial rotatory subluxation with injury of transverse ligament.

A retrospective case series. To describe a novel intraoperative reduction technique applying C1 transposterior arch lateral mass screw combined with C2 pedicle screw and rod system for pediatric acute atlantoaxial rotatory subluxation with injury of transverse ligament and evaluate outcomes of this technique. For atlantoaxial rotatory subluxation with disruption of transverse ligament or recurrent cases, initial posterior atlantoaxial fusion is a preference. It is an important prerequisite for successful surgery to achieve satisfactory reduction of atlantoaxial articulation after preoperative cranial traction. However, the placement of cranial tractor and persistent traction may be challenging due to a pediatric patient's noncompliance and unsuitable anatomic structure. Therefore, an intraoperative way to achieve reduction and to keep solid stability with only a single procedure is needed. The consecutive patients with atlantoaxial rotatory subluxation with injury of transverse ligament, including 4 males and 1 female, underwent intraoperative reduction and fixation applying C1 posterior arch lateral mass screw combined with C2 pedicle screw and rod system during a 2-year period. The surgical technique and procedure of treatment were intensively reviewed; the clinical outcomes were retrospectively investigated in the aspects of clinical symptoms and imaging appearance. Clinical follow-ups were obtained for an average of 29.4 (range, 24-30 mo) months. The clinical and radiological follow-up indicated a complete clinical relief and satisfactory reduction, stable arthrodesis of atlantoaxial articulation. No neural and vascular impairment related to this technique were observed. The primary outcome showed under sufficient preoperative imaging measurements, C1 transposterior arch lateral mass screw combined with C2 pedicle screw and rod system that obviated preoperative cranial traction provided reliable reduction and stability of atlantoaxial articulation for atlantoaxial rotatory subluxation with injury of transverse ligament. 4.

Traumatic Pediatric Atlantoaxial Rotatory Subluxation Successfully Reduced and Discharged from the Emergency Department: A Case Report

The incidence of atlantoaxial rotatory subluxation is fairly uncommon; however it is more common to be encountered in the pediatric population. It is usually seen in children after a retropharyngeal inflammation or after a minor trauma. Children have unique anatomical features such as inherent ligamentous laxity and shallower and more horizontally oriented facet joints that allow greater freedom of motion especially at the C1-2 joint making them prone to atlantoaxial subluxation. If recognized early it can be successfully managed nonsurgically before chronic inflammatory changes affect the ligaments and joint structure of the C1-2 complex. However, it is unclear in the literature if inpatient admission is necessary for treatment. Here we present a case of atlantoaxial rotatory subluxation in a child caused by forcefully throwing a ball that was successfully reduced and subsequently discharge from the pediatric emergency department all in the same visit.

Atlantoaxial Subluxation after Pyogenic Spondylitis around the Odontoid Process

Study Design. A case report and review of the literature. Objective. The aim of this study was to describe the conservative management of pyogenic spondylitis around the odontoid process. Summary of Background Data. Atlantoaxial subluxation after pyogenic spondylitis is rare. The therapeutic approach to infection of the upper cervical spine is controversial. Methods. Medical chart and radiological images of a 76-year-old male patient were retrospectively reviewed. Radiography revealed atlantoaxial subluxation, and an abscess was seen around the odontoid process on magnetic resonance images. Intravenous antibiotics and a halo vest were used to treat the patient. We then observed the patient's conservative treatment course. Results. C-reactive protein levels returned to normal 4 weeks after administration of the intravenous antibiotics. The patient's muscle weakness also completely recovered 8 weeks after administration of the intravenous antibiotics. Because the patient was able to walk without any support, surgical treatment was not necessary. Conclusions. Pyogenic spondylitis of the upper cervical spine is a rare manifestation. Surgical or conservative treatment must be selected carefully based on the patient's symptoms. If early diagnosis and treatment can be provided to the patients, conservative treatment can be achieved.

Spectrum of Skeletal and Non-Skeletal Manifestations of Morquio A Syndrome in an Adult: A Case Report

Morquio syndrome is an autosomal recessive mucopolysaccharidosis which includes type IVA, a deficiency of the enzyme N-acetylgalactosamine-6-sulfatase and type IVB a deficiency of β-galactosidase. We report a 28-year-old Indian male patient affected by Morquio A syndrome demonstrating the characteristic musculoskeletal and nonskeletal manifestations of this disease on clinical and radiological examination. Digital x-ray of the spine, pelvis, chest and knees together with magnetic resonance imaging of the entire spinal column were done. The main abnormalities were atlantoaxial subluxation, anterior beaking of the vertebrae with thoracolumbar kyphosis, platyspondyly, hypoplasia of the odontoid process, short thorax with wide anterior posterior diameter, genu valga deformity and severe multiple degenerative changes of the hips, knees, and ankle joints. Based on clinical findings and radiological features it is possible to diagnose a case of Morquio A syndrome. Nonskeletal abnormalities may also provide key insight into the clinical diagnosis of MPS IVA. Careful and systemic approach is needed to accurately diagnose the exact type as enzymatic studies are not available in most centers. Radiological examination is crucial for assessment of the skeletal and joints changes, and the rehabilitation strategies to be followed.

Atlantoaxial rotatory fixation followed by atlantoaxial subluxation in a child with Down’s syndrome : A case successfully treated with atlanto-axial fixation

Atlantoaxial rotatory fixation followed by atlantoaxial subluxation in a child with Down’s syndrome : A case successfully treated with atlanto-axial fixation

Grisel's Syndrome Induced by Mycobacterium tuberculosis.

Grisel's syndrome is a non-traumatic subluxation of the atlantoaxial joints, which is caused by an inflammatory process in the upper neck. It is rare to find literary reports of Grisel's syndrome with an evident pathogen in a lesion. For the first time in Korea, we report a 36-year-old female with Grisel's syndrome having an atlantoaxial subluxation, which was caused by a retropharyngeal abscess secondary to pulmonary Mycobacterium tuberculosis. The patient was treated with an anti-tuberculosis regimen and was prescribed a Philadelphia collar for the control of torticollis. The result of magnetic resonance imaging (MRI) showed an improved atlantoaxial alignment, after drug treatment and immobilization. This patient was neurologically intact and free from symptomatic complaints at follow-up visit. Dynamic cervical radiograph confirmed that the atlantoaxial joints had been stable. The pathophysiology of Grisel's syndrome, along with anatomical attributes, was explained on the basis of the patient's clinical course.

Atlantoaxial Subluxation due to an Os Odontoideum in an Achondroplastic Adult: Report of a Case and Review of the Literature.

The authors report the first example of an adult achondroplastic dwarf with progressive quadriparesis secondary to atlantoaxial subluxation as a consequence of an os odontoideum. Actually, craniocervical region is a frequent site of compression and myelopathy in achondroplasia particularly in children as a result of small foramen magnum and hypertrophied opisthion. Moreover, very rarely in achondroplastic patients, coexistence of atlantoaxial instability as the sequel of os odontoideum can result in further compression of the already compromised cervicomedullary neural tissues, the scenario that has been reported only in five achondroplastic children. Herein, a 39-year-old achondroplastic male suffering such an extremely rare combination is presented. With C1-C2 screw rod instrumentation, atlas arch laminectomy, limited suboccipital craniectomy, and release of dural fibrous bands, reduction, decompression, and stabilization could be achieved properly resulting in steady but progressive recovery.

A review of canine atlantoaxial joint subluxation.

Atlantoaxial subluxation was first reported in dogs nearly fifty years ago. Since that time a better understanding of the aetiologies predisposing to joint laxity and instability has been achieved. Surgeons however are still trying to address the problems associated with stabilizing this joint which by nature is often required in small juvenile dogs. This review describes the various techniques used, discussing the associated benefits and complications thereby allowing the clinician to make an informed decision on the best treatment for the individual patient.