Child's Nervous System

, Volume 24, Issue 10, pp 1101–1108 | Cite as

Craniovertebral junction database analysis: incidence, classification, presentation, and treatment algorithms

Special Annual Issue

Abstract

Introduction

The initial treatment of bony lesions at the craniocervical junction consisted of posterior decompression with enlargement of the foramen and removal of the posterior arch of the atlas and axis vertebra. This was regardless of the site of compression. Needless to say, the results were poor when an irreducible ventral lesion at the craniocervical junction caused compression of the cervicomedullary area. It was predictable that an adverse outcome would occur in approximately 35–40% of patients treated with dorsal decompression with a fixed anterior abnormality at the craniovertebral junction. Thus, it became essential to identify the causes of failure and recognize the pathology as well as attempt to improve the treatment. The need to identify natural history led to a prospective database. This database now lists 5,300 patients who were analyzed. Of these, 2,000 were children and this has been reviewed here.

Craniovertebral abnormalities in children

Review of symptoms showed that children with craniovertebral abnormalities present with failure to thrive, weakness, basilar migraine, dysphagia, sleep apnea, scoliosis, and the cervical central cord syndrome. Torticollis was seen in the very young.

Imaging techniques and treatment for craniovertebral abnormalities

New imaging techniques were utilized as they became available. Unexplained neurological symptoms and signs associated with craniovertebral abnormalities in children required angiography which was performed with dynamic motion studies. All children with reducible craniocervical abnormalities underwent stabilization. In irreducible abnormalities, decompression was paramount with a fusion to be done immediately. The decompression was accomplished in the direction in which encroachment occurred.

Treatment algorithm and classification

A treatment algorithm was formulated as a result of this database and a classification of the craniovertebral abnormalities; that holds good for the entire spectrum.

Keywords

Craniocervical junction Spinal cord compression Klippel–Feil syndrome Chiari malformation 

References

  1. 1.
    Barucha EP, Dastur HM (1964) Craniovertebral anomalies. Brain 87:469–480CrossRefGoogle Scholar
  2. 2.
    Bell C (1830) The nervous system of the human body. Longman and Co., London, pp 403–406 Ch 118Google Scholar
  3. 3.
    Chamberlain WE (1938/1939) Basilar impression (platybasia). Yale J Biol Med 11:487Google Scholar
  4. 4.
    Dastur DK, Wadia NH, Desai AD, Sinh G (1965) Medullospinal compression due to atlanto-axial dislocation and sudden haematomyelia during decompression. Brain 88:897–924PubMedCrossRefGoogle Scholar
  5. 5.
    Fang HSY, Ong AB (1962) Direct anterior approach to the upper cervical spine. J Bone Jt Surg (AM) 44:1588–1604Google Scholar
  6. 6.
    Ford FR (1952) Syncope, vertigo and disturbance of vision resulting from intermittent obstruction of the vertebral arteries due to defect in the odontoid process and excessive mobility of the second cervical vertebra. Bull Johns Hopkins Hosp 91:168–173PubMedGoogle Scholar
  7. 7.
    Ganguly DN, Roy KK (1964) A study on the craniovertebral joint in the man. Anat Anz 114:433–452PubMedGoogle Scholar
  8. 8.
    Gladstone J, Erickson-Powell W (1914–1915) Manifestation of occipital vertebra and fusion of atlas with occipital bone. J Anat Physiol 49:190–199Google Scholar
  9. 9.
    Greenberg AD (1930) Atlanto-axial dislocations. Brain 91:655–684 1968CrossRefGoogle Scholar
  10. 10.
    Grisel P (1930) Enucleation de l’atlas et torticollis nasopharyngien. Presse Med 38:50–56 ParisGoogle Scholar
  11. 11.
    Lanier RR Jr (1939) Anomalous cervico-occipital skeleton in man. Anat Rec 73:189–207CrossRefGoogle Scholar
  12. 12.
    List CF (1941) Neurologic syndromes accompanying developmental anomalies of occipital bone, atlas and axis. Arch Neurol Psychiat 45:577–616Google Scholar
  13. 13.
    Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): Data base analysis. Pediatr Neurosurg 23:260–269PubMedCrossRefGoogle Scholar
  14. 14.
    Menezes AH (1996) Rheumatological disorders. In: Menezes AH, Sonntag VKH (eds) Principles of spinal surgery. McGraw-Hill, New York, pp 705–722Google Scholar
  15. 15.
    Menezes AH (2003) Acquired abnormalities of the craniovertebral junction. In: Winn HR (ed) Youman’s neurological surgery. Saunders, Philadelphia, pp 4569–4585Google Scholar
  16. 16.
    Menezes AH (2005) Conception to implication: craniocervical junction database and treatment algorithm. Clin Neurosurg 52:154–162PubMedGoogle Scholar
  17. 17.
    Menezes AH, Graf CJ, Hibri N (1980) Abnormalities of the craniovertebral junction with cervicomedullary compression. Child’s Brain 7(l):15–30PubMedCrossRefGoogle Scholar
  18. 18.
    Menezes AH, VanGilder JC, Clark CR, el-houry G (1985) Odontoid upward migration in rheumatoid arthritis. An analysis of 45 patients with cranial settling. J Neurosurg 63:500–509PubMedGoogle Scholar
  19. 19.
    Parke WW (1978) The vascular relations of the upper cervical vertebrae. Orthop Clin North Amer 9:879–889Google Scholar
  20. 20.
    Robertson SC, Menezes AH (1998) Occipital calvarial bone graft in posterior occipitocervical fusion. Spine 23:249–255PubMedCrossRefGoogle Scholar
  21. 21.
    Ryken TC, Menezes AH (1999) Inflammatory bowel disease and the craniocervical junction. Neurosurg Focus 6(6):e10PubMedCrossRefGoogle Scholar
  22. 22.
    Sawin PD, Menezes AH (1998) A comparative analysis of fusion rates and donor site morbidity for autogenic rib and iliac crest bone grafts in posterior cervical fusions. J Neurosurg 88:255–265PubMedCrossRefGoogle Scholar
  23. 23.
    Schiff DCM, Parke WW (1972) The arterial blood supply of the odontoid process (Dens). Anat Rec 172:399–400Google Scholar
  24. 24.
    Selecki BR (1969) The effects of rotation of the atlas on the axis. Experimental work. Med J Aust 1:1012PubMedGoogle Scholar
  25. 25.
    VanGilder JC, Menezes AH, Dolan K (1987) Textbook: craniovertebral junction abnormalities. Futura, Mount Kisco, pp 1–255Google Scholar
  26. 26.
    Wadia NH (1967) Myelopathy complicating congenital atlanto-axial dislocation (A study of 28 cases). Brain 90:449–474PubMedCrossRefGoogle Scholar
  27. 27.
    Zunkeler B, Schelper R, Menezes AH (1996) Periodontoid calcium pyrophosphate dihydrate deposition disease: “pseudogout” mass lesions of the craniocervical junction. J Neurosurg 85:803–809PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Department of NeurosurgeryUniversity of Iowa Hospitals and ClinicsIowa CityUSA

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