II. Epidemiology
- Primarily occurs in children with risk factors (see below)
- May also occur in adults
III. Pathophysiology
- Minor Trauma or inflammation from mild Upper Respiratory Infection results in Cervical Ligamentous Instability
- Atlantoaxial joint becomes unstable, allowing for subluxation C1 on C2
IV. Risk Factors
- See Atlantoaxial Instability
- Juvenile Rheumatoid Arthritis
- Down Syndrome
- Marfan Syndrome
- Osteogenesis imperfecta
- Rickets
- Ehlers-Danlos
V. Signs
- Patient cannot assume a neutral head and neck position
- Head in cock-robin position
- Head with lateral flexion to one side
- Neck rotated to the opposite side
- Neck slightly flexed
VI. Imaging
- CT Cervical Spine (preferred)
- First-line study (replaces XRay)
- Dynamic CT is preferred
- When subluxed, C1 and C2 will rotate together in tandem
- First CT with head facing forward
- Next CT with head and neck maximally rotated right
- Next CT with head and neck maximally rotated left
- XRay Cervical Spine
- Indicated where CT is not available
VII. Classification
- Class 1
- Unilateral facet subluxation <3 mm
- No anterior displacement
- Intact transverse ligament
- Class 2
- Unilateral facet subluxation 3-5 mm
- Injury to transverse ligament may be present
- Class 3
- Bilateral facet subluxation >5 mm
- Risk of neurologic injury and sudden death (uncommon)
- Class 4
- Posterior displacement of axis
- Risk of neurologic injury and sudden death (uncommon)
VIII. Management
- Consult pediatric orthopedics or Spine Surgery
- Class 1 and 2 with early presentation (within 1 week)
- Conservative therapy (Analgesics, soft collar)
- Often reduces spontaneously once inflammation subsides
- May be observed outpatient with close follow-up in most cases
- Class 3 to 4 OR delayed presentation 1 to 4 weeks
- Admit to hospital on Analgesics and Muscle relaxants
- Halter traction (via chin and head straps)
- Consider manipulation under Anesthesia (OR) in refractory cases (not reducing on Halter traction)
- Manipulation under fluoroscopy
- Long traction (halo device) for 3 months after reduction
- Refractory cases (esp. late presentations >4 weeks)
- May try methods as above
- May require C1-2 fusion in refractory cases
IX. Prognosis
- Best prognosis with Class 1-2 and with early presentation (within 1 week)
X. References
- Jhun, Grock, Ebenezer in Herbert (2016) EM:Rap 16(7): 11-3