Craniovertebral junction anomalies

Craniovertebral junction (CVJ) anomalies can be congenital, developmental or due to malformation secondary to any acquired disease process. These anomalies can lead to cranial nerve compression, vertebral artery compression and obstructive hydrocephalus.


The craniovertebral junction is formed by the occipital condyles, atlas (C1), axis (C2) vertebrae and their articulations. Any process which can give rise to malformation of these structures, may result in CVJ anomaly. It can be due to congenital, developmental or acquired cause. 

On this basis of etiology, CVJ malformations can be classified as:

Congenital anomalies and malformations
Malformations of the occipital sclerotome
Malformations of atlas vertebra
Malformation of axis and odontoid process
  • atlantoaxial fusion
  • persistent ossiculum terminale
  • os odontoideum
  • odontoid dysplasia
Developmental and acquired malformations
Malformations at foramen magnum

Atlantoaxial instability

Article information

rID: 31257
Section: Gamuts
Tag: pm, pm
Synonyms or Alternate Spellings:
  • Craniovertebral junction anomaly
  • Craniocervical junction anomalies
  • Craniocervical junction anomaly
  • Craniocervical junction abnormalities
  • Craniovertebral junction abnormalities

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Cases and figures

  • Case 1: achondroplasia
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  • Case 2: atlanto-occipital assimilation
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