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.
Pathology
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 a CVJ anomaly. It can be due to a 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
remnants of proatlas
occipital condylar hypoplasia
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condylar dysplasia
segmentation anomaly of the clivus
Malformations of atlas vertebra
bifid arches
aplasia and hypoplasia of the atlas
Malformation of axis and odontoid process
atlantoaxial fusion
odontoid dysplasia
Developmental and acquired malformations
Malformations at the foramen magnum
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stenosis of the foramen magnum
paramesial invagination
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infections
trauma
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inborn errors of metabolism
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neoplastic
osteofibroma
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other
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