Atlantoaxial Instability Treatment & Management
- Author: Daxes Banit, MD; Chief Editor: Mary Ann E Keenan, MD more...
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.
- 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.
- 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.
Surgical Care
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 individuals with rotatory dislocation, the treatment for traumatic injuries is a collar for type I injuries that are regarded as stable subluxations. Type II injuries may be potentially unstable. Type III and IV rotatory displacements that are unstable are treated surgically with a reduction and C1-2 fusion. The techniques of fusion vary from sublaminar wiring techniques like Brooks or Gallie, Halifax clamp, or transarticular screw of Magerl.[17, 18, 19, 20, 21, 5, 22, 23, 24] In situations of associated injuries, extending the fusion to the occiput may be required.
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.
- Posterior cervical spinal fusion can successfully treat symptomatic AAI in many cases.[25, 26] 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.[27]
- 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.
- Usually, because of severe osteoporosis in patients with RA, multiple pins are used to improve fixation.
- In addition, the nature of the subaxial cervical spine should be considered when planning the distal extent of the fusion.
Consultations
- Neurologist
- Neurosurgeon
- Orthopedic surgeon
Activity
- Before participation in sports, evaluations are recommended to detect neurologic involvement.
- Spinal cord compression can arise or worsen if susceptible patients are subjected to extreme ranges of motion. Special care should be taken to avoid excessive flexion or extension of the neck.
- Special Olympics, Inc., currently requires that all children with Down syndrome who compete in Special Olympics games have radiographic and neurologic examinations to exclude AAI. Individuals with AAI are restricted from participation in certain activities that may result in cervical spine injury. These include gymnastics, diving, pentathlon, butterfly stroke, diving starts in swimming, high jump, soccer, and certain warm-up exercises.[28]
- In 1983, the American Academy of Pediatrics Committee on Sports Medicine and Fitness issued a statement in agreement with the recommendations and requirements of the Special Olympics. However, a review of evidence in 1995 caused the committee to rescind their recommendations that all children with Down syndrome should be screened radiographically.[29]
- Although no indication exists that children with asymptomatic AAI are at increased risk of subluxation or progression to symptomatic AAI or that routine screening by radiographs is necessary, recommendations vary.
Milz S, Schluter T, Putz R, et al. Fibrocartilage in the transverse ligament of the human atlas. Spine. Aug 15 2001;26(16):1765-71. [Medline].
Parke WW, Rothman RH, Brown MD. The pharyngovertebral veins: an anatomical rationale for Grisel''s syndrome. J Bone Joint Surg Am. Apr 1984;66(4):568-74. [Medline].
Boden SD, Dodge LD, Bohlman HH, Rechtine GR. Rheumatoid arthritis of the cervical spine. A long-term analysis with predictors of paralysis and recovery. J Bone Joint Surg Am. Sep 1993;75(9):1282-97. [Medline].
Clark CR, Goetz DD, Menezes AH. Arthrodesis of the cervical spine in rheumatoid arthritis. J Bone Joint Surg Am. Mar 1989;71(3):381-92. [Medline].
Chun HJ, Oh SH, Yi HJ, Ko Y. Efficacy and durability of the titanium mesh cage spacer combined with transarticular screw fixation for atlantoaxial instability in rheumatoid arthritis patients. Spine (Phila Pa 1976). Oct 15 2009;34(22):2384-8. [Medline].
Babic-Naglic D, Potocki K, Curkovic B. Clinical and radiological features of atlantoaxial joints in rheumatoid arthritis. Z Rheumatol. Aug 1999;58(4):196-200. [Medline].
Mikulowski P, Wollheim FA, Rotmil P. Sudden death in rheumatoid arthritis with atlanto-axial dislocation. Acta Med Scand. Dec 1975;198(6):445-51. [Medline].
Yamazaki M, Someya Y, Aramomi M, Masaki Y, Okawa A, Koda M. Infection-related atlantoaxial subluxation (Grisel syndrome) in an adult with Down syndrome. Spine. Mar 1 2008;33(5):E156-60. [Medline].
Rana NA, Hancock DO, Taylor AR, Hill AG. Upward translocation of the dens in rheumatoid arthritis. J Bone Joint Surg Br. Aug 1973;55(3):471-7. [Medline].
Pueschel SM. Should children with Down syndrome be screened for atlantoaxial instability?. Arch Pediatr Adolesc Med. Feb 1998;152(2):123-5. [Medline].
Berkowitz ID, Raja SN, Bender KS, Kopits SE. Dwarfs: pathophysiology and anesthetic implications. Anesthesiology. Oct 1990;73(4):739-59. [Medline].
Kulkarni AG, Goel AH. Vertical atlantoaxial index: a new craniovertebral radiographic index. J Spinal Disord Tech. Feb 2008;21(1):4-10. [Medline].
Ilkko E, Tikkakoski T, Pyhtinen J. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia. Eur J Radiol. Nov 1998;29(1):55-60. [Medline].
Yuksel M, Heiserman JE, Sonntag VK. Magnetic resonance imaging of the craniocervical junction at 3-T: observation of the accessory atlantoaxial ligaments. Neurosurgery. Oct 2006;59(4):888-92; discussion 892-3. [Medline].
Hung SC, Wu HM, Guo WY. Revisiting Anterior Atlantoaxial Subluxation with Overlooked Information on MR Images. AJNR Am J Neuroradiol. Dec 31 2009;[Medline].
Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am. Jan 1977;59(1):37-44. [Medline].
Haid RW Jr. C1-C2 transarticular screw fixation: technical aspects. Neurosurgery. Jul 2001;49(1):71-4. [Medline].
Naseer R, Bailey SI. Atlantoaxial instability treated with transarticular screw fixation. Int Orthop. 2001;25(4):268-71. [Medline].
Henriques T, Cunningham BW, Olerud C, et al. Biomechanical comparison of five different atlantoaxial posterior fixation techniques. Spine. Nov 15 2000;25(22):2877-83. [Medline].
Sugimoto Y, Tanaka M, Nakanishi K, Misawa H, Takigawa T, Ozaki T. Assessing the range of cervical rotation in patients with rheumatoid arthritis after atlantoaxial screw fixation using axial CT. Spine. Oct 1 2007;32(21):2318-21. [Medline].
Gunnarsson T, Massicotte EM, Govender PV, Raja Rampersaud Y, Fehlings MG. The use of C1 lateral mass screws in complex cervical spine surgery: indications, techniques, and outcome in a prospective consecutive series of 25 cases. J Spinal Disord Tech. Jun 2007;20(4):308-16. [Medline].
Lee SH, Kim ES, Sung JK, Park YM, Eoh W. Clinical and radiological Comparison of treatment of atlantoaxial instability by posterior C1-C2 transarticular screw fixation or C1 lateral mass-C2 pedicle screw fixation. J Clin Neurosci. Apr 15 2010;[Medline].
Li WL, Chi YL, Xu HZ, Wang XY, Lin Y, Huang QS, et al. Percutaneous anterior transarticular screw fixation for atlantoaxial instability: a case series. J Bone Joint Surg Br. Apr 2010;92(4):545-9. [Medline].
Elgafy H, Potluri T, Goel VK, Foster S, Faizan A, Kulkarni N. Biomechanical analysis comparing three C1-C2 transarticular screw salvaging fixation techniques. Spine (Phila Pa 1976). Feb 15 2010;35(4):378-85. [Medline].
Ahmed R, Traynelis VC, Menezes AH. Fusions at the craniovertebral junction. Childs Nerv Syst. Apr 4 2008;[Medline].
Claybrooks R, Kayanja M, Milks R, Benzel E. Atlantoaxial fusion: a biomechanical analysis of two C1-C2 fusion techniques. Spine J. Nov-Dec 2007;7(6):682-8. [Medline].
Castel E, Benazet JP, Samaha C, et al. Delayed closed reduction of rotatory atlantoaxial dislocation in an adult. Eur Spine J. Oct 2001;10(5):449-53. [Medline].
Hankinson TC, Anderson RC. Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. Mar 2010;66(3 Suppl):32-8. [Medline].
American Academy of Pediatrics Committee on Sports Medicine & Fitness. Atlantoaxial instability in Down syndrome: subject review. Pediatrics. Jul 1995;96(1 Pt 1):151-4. [Medline].
Morton RE, Khan MA, Murray-Leslie C, Elliott S. Atlantoaxial instability in Down's syndrome: a five year follow up study. Arch Dis Child. Feb 1995;72(2):115-8; discussion 118-9. [Medline].

