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ATLANTOAXIAL INSTABILITY

Abhra 9/08/2011

Saggital view of the neck showing anatomy of the cervical spine.The odontoid sits just behind the anterior arch of atlas and is limited posteriorly by the transverse ligament of the atlas

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physiologically decreasing opening between these 2 segments. and free space for cord are each about 1 cm in diameter. • Steele's Rule of Thirds: .spinal canal of the atlas is large compared w/ that of other segments.• Atlanto-Axial Articulation: . around laterally central but anteriorly eccentric odontoid process.approx 50 % of cervical rotation takes place between atlas and axis.spinal cord.canal of atlas is about 3 cm in its AP diameter. .lateral wall of atlas rotates to across canal of axis. which rotation around axis along w/ translational displacement without pressure on the spinal cord.anterior displacement of the atlas that exceeds one centimeter may jeopardize the adjacent segment of the spinal cord . odontoid process. . . .

anteroposterior movement .  Children-increase slightly on flexion ◦ Adults-unchanged b/w flexion & extension  Transverse ligament -primary restraint against atlantoaxial. an atlantodens interval (ADI. distance between odontoid process and the posterior border of the anterior arch of the atlas) of greater than 3 mm in adults and of greater than 5 mm in children as measured on plain radiography.

• Atlantoaxial instabilitypredentate space >3 mm in adults > 5 mm in children • Symptoms present -atlas impinge on the spinal cord .

 C1-C2 subluxation: Types ◦ Anterior ◦ Lateral ◦ Posterior ◦ Atlantoaxial impaction (AAI) .

Etiology • . .Morquio syndrome.Achondroplasia: . .Grisel's syndrome: .Down syndrome (25% of patients).hyperemia causes demineralization of attachment of transverse ligament to anterior arch of atlas.Spondyloepiphyseal dysplasia: .Rheumatiod Arthritis (adults). .Klippel Feil. .atlantoaxial instability may be noted in nl child in association w/ pharyngeal infection (Grisel's syndrome).Larsen's syndrome: -Neurofibromatosis -Osteogenesis imperfecta -Spondyloarthopathy . with subsequent rotary subluxation of atlas on axis or anterior atlantoaxial subluxation. . .

AAD in RA • • • • subluxation can occur in up to 70% of patients with rheumatoid arthritis. - . (as noted by T. among patients that develop myelopathy. approximately 11% of rheumatoid arthritis patients will develop cord compression from atlantoaxial subluxation. but frank dislocation occurs in about 25%. • anterior instability is much more common than posterior instability and occurs more often in men. 5 years survival rate is 80% but the 10 year survival rate is 28%. C1-C2 articulation is synovial which accounts for its frequent involvement in RA. Mori MD et al 1998).

w/ C-spine flexion.stretching and destruction of these structure allows atlas vertebra to move forward relative to the axis. C1.corticosteroid use.spinal cord being compressed between posterior arch of the atlas and the odontoid peg. . & transverse ligament. .erosive and deforming disease. • transverse ligament elongation and rupture: . dens or both. resulting in destruction of transverse ligament. . . atlas moves forward relative to axis.RA nodules. .seropositivity. .• • etiology: results from pannus formation at synovial joints between dens. • • • risk factors: . dens erosion: in some cases odontoid is totally eroded by inflammatory reaction.

8m) lateral views in flexion &extension .Radiology  Views ◦ anteroposterior (AP) ◦ AP “open mouth” odontoid ◦ 6-ft (1.

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lateral masses of Cl positioned more than 2 mm laterally with relation to C2 .5mm • Lateral subluxation frontal view .• Anterior subluxation Predentate space> 2.

• Posterior subluxation posterior aspect of anterior arch of atlas posterior to the anterior border of the body of C2 .

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Preodontoid space measures 8 mm.5 -3mm .Normal 2.Posterior dislocation of the odontoid process.

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odontoid tip in relation to McGregor’s(MG)*  8 mm above MG in men  9. Atlantoaxial impaction: ◦ settling of the skull onto Cl & Cl onto C2 ◦ Erosion & bone loss at intervening joints ◦ Lateral views.7 mm above in women .

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.CLINICAL FEATURES • Earliest and most common symptom of cervical subluxation is pain radiating superiorly towards the occiput . including painless sensory loss in the hands or feet. Additional symptoms of subluxation include: • Spastic quadriparesis which is slowly progressive • Sensory findings are also common.

• Neurologic findings in patients with vertical atlantoaxial subluxation may also include decreased sensation in the distribution of the fifth cranial nerve.• In patients with C1-C2 subluxation. sensory loss in the C2 area. and pyramidal lesions . transient episodes of medullary dysfunction (such as respiratory irregularity) associated with vertical penetration of the odontoid process of C2 and probable vertebral artery compression . nystagmus. Movement of the hands in this setting may result in paresthesias in the shoulder or arms.

convulsions.symptoms of spinal cord compression that demand immediate attention and intervention include [20]: • A sensation of the head falling forward upon flexion of the cervical spine • Changes in levels of consciousness • "Drop" attacks • Loss of sphincter control • Respiratory dysfunction • Dysphagia. hemiplegia. or nystagmus • Peripheral paresthesias without evidence of peripheral nerve disease or compression . vertigo. dysarthria.

non ambulatory .Neurological impairment Ranawat class I II Neurological status No neurological deficit Subjective weakness with hyperreflexia and dysesthesia Objective weakness and long tract signs. ambulatory IIIA IIIB Objective weakness and long tract signs.

SIGNS • Loss of occipitocervical lordosis • Resistance to passive spine motion • Abnormal protrusion of the axial arch felt by the examining finger on the posterior pharyngeal wall • In addition. neurologic findings appropriate to the symptoms described above may be seen. including: • Increased deep tendon reflexes • Extensor plantar responses • Muscle weakness. or muscle atrophy • Gait disorders . spasticity.

the spinal cord. particularly in patients with evidence of upper cervical cord or brainstem compression or subaxial myelopathy • Bone marrow edema (BME) can be observed by MRI in patients with early cervical spine involvement. because it permits visualization of the pannus producing cord compression. with involvement of the odontoid process in patients with changes at the atlantoaxial level and involvement of the vertebral plates and the interapophyseal joints subaxiall .IMAGING-MRI • particularly valuable in the assessment of cervical spine disease in RA. and bone • development of neurological dysfunction is strongly associated with MRI evidence of atlantoaxial spinal canal stenosis.

unless flexion and extension MR images document excessive subluxation. in the worst case the measured distance differed by 7 mm. the measured atlantoaxial subluxation by MRI was less than that noted on radiographs in 19 of the 23 patients. plain film radiography is still needed to assess atlantoaxial stability .• one draw-back of MRI imaging is that it often underestimates the degree of atlantoaxial subluxation when compared to plain film radiography This was illustrated in a series of 23 patients with RA or JIA who had both radiographs and MRI with flexion and extension views performed within a one-month time frame [36]. After accounting for magnification on the plain films. • • Thus.

• Since neck positioning required for intubation prior to surgery may be fatal among patients with RA and unrecognized C1-C2 disease. and since subluxation is not always symptomatic. radiographic evaluation of the cervical spine should also be considered for all patients with RA scheduled to undergo surgery requiring manipulation of the neck for either anesthesia or surgery .

NATURAL HISTORY • Patients with subluxation and signs of spinal cord compression have a poor prognosis without surgery. in a study of 21 patients with atlantoaxial subluxation and signs of myelopathy who were medically managed medically. neurologic deterioration occurred in 16 of 21 (76 percent) and all were unable to walk within three years of follow up . None survived more than eight years • . • In this setting. myelopathy progresses rapidly and death may quickly ensue As an example.

• MRI findings may be more helpful than plain film radiography in determining prognosis. • As an example. • Among all patients. with only 18 percent worsening with time. two due to pain only. 60 percent deteriorated with conservative management over a median of 12 months . nine eventually required surgical intervention: six due to a combination of pain and progressive neurologic deficits. • Those with subaxial cord compression fared better. and one because of painless neurologic deterioration . among 82 patients with MRI evidence of cord compression at the level of C1-C2.

• 195 patients with RA of recent onset (two years or less) were randomly assigned to a regimen of sulfasalazine. but in none of those receiving combination therapy after two years of treatment. the occurrence of anterior atlantoaxial subluxations was significantly associated with initial single DMARD therapy . methotrexate hydroxychloroquine and prednisolone or to sulfasalazine alone [48].PREVENTION • Limited evidence suggests that the administration of combination therapy consisting of disease modifying anti-rheumatic drugs may help prevent the development of cervical spine subluxation. DMARD treatment was unrestricted after two years • At five years of follow-up. respectively. • Atlantoaxial impaction or anterior subluxation developed in 2 and 7 percent of the sulfasalazine alone group.

TREATMENT • Medical • surgical .

These include small falls. • Spinal manipulation is contraindicated . • stiff cervical collars should be prescribed for stability • Collars that are not rigid (and therefore more comfortable for the patient) give reassurance to both physician and patient. whiplash injuries. but provide little structural support. and intubation.MEDICAL • Patients with severe subluxation but without signs of cord compression are at risk for severe injury and perhaps death due to a variety of insults.

and other important patient outcomes were not assessed in this study. the efficacy of neck flexor muscle strengthening for symptoms related to subluxation. isometric neck extensor muscle tightening worsened radiographically apparent atlantoaxial subluxation in those with unstable articulations. isometric exercise of the neck extensors should be avoided • • • . A decrease in anterior atlantoaxial subluxation was noted in a subgroup of seven patients with RA and unstable atlantoaxial joints during active isometric neck flexor muscle contraction While this suggests that isometric neck flexor exercise is probably safe. while further investigation of neck flexor strengthening may be warranted. radiographic progression. Thus.PHYSIOTHERAPY • • role of neck muscle strengthening exercises is uncertain. In contrast to the neck flexors.

• Patients with pain due to irritation of C2 nerve root. . but who do not have evidence of cord compression. may benefit from agents used for chronic neuropathic pain. although the relief is generally temporary. • These patients may obtain some benefit from local nerve blocks.

and a decreased risk of neurologic progression. an improvement in myelopathy in some patients.SURGERY • Benefits offered by surgical management of patients with atlantoaxial subluxation who have myelopathy include an improved survival.and ten-year survival rates for those who were operated upon were 84 and 37 percent. none of the 21 patients managed conservatively survived more than eight years. • The beneficial effects of surgery were illustrated in an observational study that compared 19 patients with symptomatic atlantoaxial subluxation who underwent laminectomy and occipitocervical fusion with those of 21 others who were managed conservatively • The five. while in the nonoperative group. respectively. • In contrast. • Neurologic improvement was noted in 68 percent following surgery. 76 percent had neurologic deterioration .

.sublaminar wiring may be contra-indicated in these patients when the SAC is less than 12 mm.ADI of > 7 to 10 mm or posterior space (SAC) < 13 mm is contraindication surgery in other areas of body & C-spine should be stabilized first. .if myelopathy is present. .if there is an associated irreducible atlanto-axial dislocation then consider additional decompressive laminectomy of the atlas . this may be the procedure of choice. this pannus tissue will often resolve.in RA.more than 9 mm of anterior atlantoaxial subluxation places pt at high risk for development of cord compression.most indicated for patients w/ C1/C2 subluxation which is reducible. .after posterior cervical fusion.results may be unacceptable if myelopathy is present. .• • • indications for surgery: . ATLANTOAXIAL FUSION . periodontoid pannus tissue is often present and can contribute to cord compression. . OCCIPITOCERVICAL FUSION .

.COMPLICATIONS • surgery is less successful in patients w/ servere Ranawat IIIb lesions (non ambulatory with objective weakness).complications include pseudoarthrosis & recurring myelopathy.pseudoarthrosis rate can be decreased by extending fusion to occiput with wire fixation . .

Supplement 1. Volume 99. 111-111(1) . July 1997 . pp.NEWER SURGERIES • Stereotactically-guided C1-2 transarticular screw placement Clinical Neurology and Neurosurgery.

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