Atlantoaxial Instability Clinical Presentation

  • Author: Daxes Banit, MD; Chief Editor: Mary Ann E Keenan, MD   more...
 
Updated: May 13, 2010
 

History

  • In some persons with Grisel syndrome, an antecedent illness or infection occurs.[8]
  • In cases associated with trauma, head and facial injuries may be present. The head striking the windshield or the dashboard often leads to AAI.
  • 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.
  • In individuals with Morquio syndrome, AAI is usually secondary to odontoid hypoplasia or aplasia. These persons tend to present later in childhood and usually later than those with spondyloepiphyseal dysplasia congenita.
  • 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.
  • AAI also is observed in persons with the rare syndrome metatropic dysplasia. These children survive into young adulthood. This condition can be confused with Kniest syndrome in infancy. Children undergoing other procedures should have thorough neck examinations.
  • 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.
  • Predisposing factors for AAI are discussed in Causes.
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Physical

  • Neurologic examination
    • A careful neurologic examination should be conducted, especially for children at risk.
    • Assess sensory, motor, and other neurologic functions.
    • Upper motor neuron signs, including hyperreflexia, clonus, and extensor plantar reflexes, may be indicative of symptomatic AAI.
    • 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. Rana reported the subtle association of involvement of the fifth cranial nerve, which has a descending tract that extends to C2.[9] 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.
  • Persons with AAI due to inflammatory processes less frequently exhibit signs of root or cord involvement.
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Causes

  • AAI associated with bony abnormalities can be caused by abnormal development or ossification of the odontoid or fracture with or without remodeling.
  • Tumors can cause a pathologic fracture of the body of the axis, resulting in AAI.
  • The cause of AAI associated with problems of the transverse ligament is not known. An abnormal protein structure in connective tissue may cause the ligamental laxity observed in individuals with Down syndrome. Inflammation of the ligament, as observed in persons with AAI secondary to infections or RA, can weaken the joint and predispose to subluxation.
  • The following conditions can be associated with AAI:
    • Down syndrome[10]
    • Congenital scoliosis
    • Osteogenesis imperfecta
    • Neurofibromatosis
    • Morquio syndrome
    • Larsen syndrome
    • Spondyloepiphyseal dysplasia congenita
    • Chondrodysplasia punctata
    • Metatropic dysplasia (a rare syndrome that can present with AAI)
    • Kniest syndrome (also can present with AAI; a workup must be performed prior to any operative interventions)
    • Odontoid abnormalities
    • Os odontoideum
    • Ossiculum terminale
    • Third condyle
    • Hypoplasia or absence of the dens
    • Dwarfism[11]
    • Pseudoachondroplasia
    • Cartilage-hair hyperplasia
    • Rheumatoid arthritis
    • Ankylosing spondylitis
    • Scott syndrome
    • Infections of the head and neck
    • Tumors
    • Trauma
    • Cerebral palsy
    • Steroid therapy
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Contributor Information and Disclosures
Author

Daxes Banit, MD  Spine Fellow, Charlotte Orthopedic Specialists, PA

Daxes Banit, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel Murrey, MD  Clinical Faculty, Department of Orthopedic Surgery, Carolinas Medical Center; Consulting Surgeon, Presbyterian Orthopedic Hospital; Co-President, OrthoCarolina

Daniel Murrey, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Physician Executives, American Medical Association, North American Spine Society, and North Carolina Medical Society

Disclosure: Nothing to disclose.

Bruce Darden II, MD  Director, Spine Surgery Fellowship, Charlotte Spine Center

Bruce Darden II, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, North American Spine Society, North Carolina Medical Society, Scoliosis Research Society, Southern Medical Association, and Southern Orthopaedic Association

Disclosure: Synthes Consulting fee Speaking and teaching; Synthes Grant/research funds Other; Synthes fellowship support Other; Stryker Royalty Other; Stryker Consulting fee Consulting; Orthovita Ownership interest Consulting

Specialty Editor Board

James F Kellam, MD  Vice-Chair, Department of Orthopedic Surgery, Director of Orthopedic Trauma and Education, Carolinas Medical Center

James F Kellam, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Orthopaedic Trauma Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

William O Shaffer, MD  Professor, Vice-Chairman and Residency Program Director, Department of Orthopedic Surgery, University of Kentucky at Lexington

William O Shaffer, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, International Society for the Study of the Lumbar Spine, Kentucky Medical Association, Kentucky Orthopaedic Society, North American Spine Society, Southern Medical Association, and Southern Orthopaedic Association

Disclosure: DePuySpine 1997-2007 (not presently) Royalty Consulting; DePuySpine 2002-2007 (closed) Grant/research funds SacroPelvic Instrumentation Biomechanical Study; DePuyBiologics 2005-2008 (closed) Grant/research funds Healos study just closed; DePuySpine 2009 Consulting fee Design of Offset Modification of Expedium

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Mary Ann E Keenan, MD  Professor, Vice Chair for Graduate Medical Education, Department of Orthopedic Surgery, University of Pennsylvania School of Medicine; Chief of Neuro-Orthopedics Program, Department of Orthopedic Surgery, Hospital of the University of Pennsylvania

Mary Ann E Keenan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, American Society for Surgery of the Hand, and Orthopaedic Rehabilitation Association

Disclosure: Nothing to disclose.

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The transverse ligament holds the dens against the anterior arch of the atlas.
A midsagittal section of the upper cervical spine. Note the landmarks for measuring the anterior atlantodens interval and the posterior atlantodens interval.
The 4 types of atlantoaxial rotatory subluxation.
 
 
 
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