Atlantoaxial Instability

Excessive movement at the junction between the atlas (C1) and  

.axis (C2) as a result of either a bony or ligamentous abnormality

The causes of AAI are varied. AAI sometimes results from trauma.
Other cases occur secondary to an upper respiratory infection or
infection following head and neck surgery. Another cause is
rheumatoid arthritis (RA), with its predilection for the upper cervical
spine. In addition, congenital anomalies, syndromes, or metabolic
.diseases can increase the risk of AAI

:Patho-physiology

The 3 patterns of AAI are flexion-extension, distraction, and  

rotation. The most common abnormalities involve the transverse
ligament or odontoid process.[1] The strong transverse ligament and
the facet capsules maintain the integrity of the atlantoaxial
articulation. The transverse ligament is the primary restraint
against atlantoaxial anteroposterior (AP) translation. Cadaveric
studies have demonstrated that the transverse ligament is stronger
.in resisting AP forces before failure versus lateral forces

Certain congenital conditions can be associated with AAI.

Associated conditions include Down syndrome, congenital
scoliosis,osteogenesis imperfecta,neurofibromatosis, Morquio
syndrome, Larsen syndrome, spondyloepiphyseal dysplasia
congenita, chondrodysplasia punctata, metatropic dysplasia, and
Kniest syndrome.
 In individuals with Down syndrome, the primary cause of AAI is the
laxity of the transverse ligament, which holds the dens against the
posterior border of the anterior arch.
Presentation

 the cervical spine is involved more frequently in patients with

RA. Cervical involvement tends to be asymptomatic in the scenario
of systemic rheumatoid manifestations. In addition, the severity of
the systemic manifestations should serve as a marker of the
degree of cervical involvement.
 Two forms of spondyloepiphyseal dysplasia exist. The
congenita form is associated with a 40% risk of AAI. The tarda
form usually does not manifest AAI.
 The primary cause of disability and death is AAI in
individuals with chondrodysplasia punctata. Death from spinal cord
compression can occur at an early age.

 Usually, persons with congenital anomalies do not become

symptomatic before the third decade of life. The spine is assumed
to be able to accommodate differing regions of hypermobility and
fusions. With time, the degenerative changes occurring in the
lower cervical spine increase rigidity and alter the balance. This
gradual loss of motion places increasing loads on the atlantoaxial
articulation.
 Physical exam:

 Neurologic examination
o A careful neurologic examination should be conducted,
especially for children at risk.
o Assess sensory, motor, and other neurologic functions.
o Upper motor neuron signs, including hyperreflexia,
clonus, and extensor plantar reflexes, may be
indicative of symptomatic AAI.
o Somatosensory evoked response may reveal
information regarding neurologic involvement.

 In individuals with rotatory displacement, a cock-robin

deformity or torticollis can be the presenting symptom.

 Many patients with RA present with occipital pain. Others

develop myelopathy, vertigo, brainstem signs, lower cranial
nerve palsies, or myelopathy.

 The brainstem findings occur with either basilar invaginations

or with the alteration of the path of the vertebral artery with
changing of normal anatomy.

 Pyramidal signs, including hyperactive reflexes, a positive

Babinski sign, and proprioceptive loss, should alert the
physician for developing myelopathy.

 The most specific physical findings in patients with

symptomatic AAI secondary to infections of the head and
 neck are torticollis, tenderness over the spinous process of
the axis with palpation, and the Sudeck sign (displacement of
spine of the axis in the direction of head tilt).

 A reduction in size of the nasopharynx and increased nasal

resonance also may be present due to forward displacement
of the arch of the atlas.

 Treatment

 Medical care:
 Unless symptoms of spinal cord compression occur, AAI
requires no treatment.

 Once symptoms arise, cervical spine stabilization is

indicated until surgical stabilization is performed.

 In persons with rotatory displacement, the time of

presentation dictates the treatment. Most of these patients'
conditions resolve spontaneously, and additional care is not
sought.
o Patients presenting with subluxation of less than a
week's duration are treated with a soft collar and rest
for a week. If close follow-up fails to document
reduction, a period of halter traction with analgesics
and muscle relaxants is warranted. If this fails, halo
bracing can be undertaken.
 o In patients with rotatory displacement for more than 1
month, a period of halo traction for 3 weeks is tried.
Usually, 2 types of patients are in this group, those
whose subluxation improves with bracing but recurs
when bracing stops and those who usually present with
a fixed deformity.

o Surgical:

The treatment goals for persons with AAI are to protect the spinal cord,
stabilize the spinal column, decompress neural tissue, and reduce any
deformity.
In C1-2 subluxation or dislocation, an ADI of greater than 3 mm indicates
injury to the transverse ligament. In most cases, the injury is purely
ligamentous and unlikely to heal. Therefore, these injuries usually are treated
with posterior C1-2 fusion. If the CT scan revealed a bony avulsion injury as
the source of failure, a trial of halo bracing may be tried.
In rotatory displacement from nontraumatic causes, the pediatric population is
most susceptible. In a patient with either a fixed deformity or recurrent
deformity despite reduction in halo brace, a posterior C1-2 fusion is indicated.
 Surgery has been demonstrated to be most successful for treating
patients with ligamentous instability but has demonstrated less success
in patients with osseous instability. Best results have been obtained in
patients with severe pain and mild myelopathy. Thus, detecting
symptoms early is preferable for most successful treatment.

 It has been demonstrated that surgery is unlikely to reverse clinical

symptoms when the spinal cord is compressed by more than 60%.
However, surgery is not recommended for individuals without spinal
cord involvement due to an unclear natural history of AAI.

 In individuals with RA, the goals of surgery are to relieve neural

compression, relieve pain, and address instability. Indications for
surgery include AAS of greater than 8 mm with evidence of cord
compression on dynamic flexion-extension view, PADI of 14 mm or
less, more than 3.5 mm of subaxial subluxation, or progressive
neurologic deficit.

o With atlantoaxial rotatory subluxation in patients with RA,

treatment comprises gradual reduction in halo traction followed
with an occiput to C2 fusion. The halo may be incorporated for
postoperative bracing.

o Usually, because of severe osteoporosis in patients with RA,

multiple pins are used to improve fixation.
o In addition, the nature of the subaxial cervical spine should be
considered when planning the distal extent of the fusion.