Atlantoaxial Instability

Aka: Atlantoaxial Instability

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II. Causes

  1. Trauma
  2. Congenital Disorders
    1. Down Syndrome (15% of those age <21 years old)
    2. Congenital Dwarfism
    3. Congenital Scoliosis
    4. Odontoid hypoplasia
    5. Osteogenesis Imperfecta
    6. Neurofibromatosis
    7. Other congenital disorders of skeletal dysplasia and other osseous abnormalities
  3. Inflammatory Conditions
    1. Rheumatoid Arthritis

III. Symptoms suggestive of Atlantoaxial Instability

  1. Gait disturbance
  2. Spasticity
  3. Change in bowel or Bladder function

IV. Signs suggestive of Atlantoaxial Instability

  1. Neck Pain
  2. Torticollis
  3. Motor weakness in upper or lower extremities
  4. Increased Deep Tendon Reflexes
  5. Babinski response
  6. Clonus

V. Radiology: C-Spine XRay

  1. Indications
    1. Routine screening at age 5-6 no longer recommended
    2. Xray indicated for Signs or symptoms above
    3. Screen prior to Contact Sport participation
  2. Views
    1. Neutral
    2. Flexion
    3. Extension
  3. Interpretation
    1. Measure C1-C2 space
      1. Posterior segment of anterior arch of C1
      2. Anterior segment of odontoid process of C2
    2. Distance should be no more than 5 mm

VI. Prognosis

  1. Precludes Contact Sport participation if present

VII. References

  1. Frost (1999) Clin Neuropathol 18(5):250-9 [PubMed]
  2. Msall (1990) Pediatrics 85:447-9 [PubMed]
  3. Pueschel (1987) Pediatrics 80:555-60 [PubMed]

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