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Craniovertebral Junction Embryology
Kevin R. Moore, MD
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Terminology

    • Abbreviations: Craniovertebral junction (CVJ)

Anatomy-Based Imaging Issues

  • Development of the skull base, atlas (C1), and axis (C2) is embryologically distinct compared to the subaxial cervical spine and therefore merits separate discussion. Composed of the occiput, atlas (C1), and axis (C2), with the adjacent muscles, soft tissues, and ligaments, the CVJ serves as the articulation between the skull base and the spine.

Imaging Protocols

  • Multiplanar MR imaging provides superior contrast resolution to other imaging modalities and is ideal for evaluation of soft tissues, neural structures, and ligaments. Conversely, multidetector CT with orthogonal reconstructions optimally defines CVJ osseous anatomy and pathology.

Embryology

  • Occipital Bone and Posterior Skull Base

    • Atlas (C1)

      • Axis (C2)

        Pathology-Based Imaging Issues

        • Occipital Bone and Skull Base Anomalies

          • Atlas (C1) Anomalies

            • Axis (C2) Anomalies

              Clinical Implications

              • Klippel-Feil Syndrome

                • Down Syndrome (Trisomy 21)

                  • Achondroplasia

                    • Mucopolysaccharidoses

                      Selected References

                      1. Sarat Chandra P: Craniovertebral junction anomalies: changing paradigms, shifting perceptions: where are we and where are we going? Neurospine. 16(2):209-11, 2019
                      2. Gaunt T et al: Abnormalities of the craniovertebral junction in the paediatric population: a novel biomechanical approach. Clin Radiol. 73(10):839-54, 2018
                      3. Lopez AJ et al: Anatomy and biomechanics of the craniovertebral junction. Neurosurg Focus. 38(4):E2, 2015
                      4. Benglis D et al: Neurologic findings of craniovertebral junction disease. Neurosurgery. 66(3 Suppl):13-21, 2010
                      5. Hankinson TC et al: Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. 66(3 Suppl):32-8, 2010
                      6. Menezes AH et al: Remnants of occipital vertebrae: proatlas segmentation abnormalities. Neurosurgery. 64(5):945-53; discussion 954, 2009
                      7. Kumar R et al: Craniovertebral junction anomaly with atlas assimilation and reducible atlantoaxial dislocation: a rare constellation of bony abnormalities. Pediatr Neurosurg. 44(5):402-5, 2008
                      8. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                      9. Smoker WR et al: Imaging the craniocervical junction. Childs Nerv Syst. 24(10):1123-45, 2008
                      10. Gholve PA et al: Occipitalization of the atlas in children. Morphologic classification, associations, and clinical relevance. J Bone Joint Surg Am. 89(3):571-8, 2007
                      Related Anatomy
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                      Related Differential Diagnoses
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                      References
                      Tables

                      Tables

                      Terminology

                        • Abbreviations: Craniovertebral junction (CVJ)

                      Anatomy-Based Imaging Issues

                      • Development of the skull base, atlas (C1), and axis (C2) is embryologically distinct compared to the subaxial cervical spine and therefore merits separate discussion. Composed of the occiput, atlas (C1), and axis (C2), with the adjacent muscles, soft tissues, and ligaments, the CVJ serves as the articulation between the skull base and the spine.

                      Imaging Protocols

                      • Multiplanar MR imaging provides superior contrast resolution to other imaging modalities and is ideal for evaluation of soft tissues, neural structures, and ligaments. Conversely, multidetector CT with orthogonal reconstructions optimally defines CVJ osseous anatomy and pathology.

                      Embryology

                      • Occipital Bone and Posterior Skull Base

                        • Atlas (C1)

                          • Axis (C2)

                            Pathology-Based Imaging Issues

                            • Occipital Bone and Skull Base Anomalies

                              • Atlas (C1) Anomalies

                                • Axis (C2) Anomalies

                                  Clinical Implications

                                  • Klippel-Feil Syndrome

                                    • Down Syndrome (Trisomy 21)

                                      • Achondroplasia

                                        • Mucopolysaccharidoses

                                          Selected References

                                          1. Sarat Chandra P: Craniovertebral junction anomalies: changing paradigms, shifting perceptions: where are we and where are we going? Neurospine. 16(2):209-11, 2019
                                          2. Gaunt T et al: Abnormalities of the craniovertebral junction in the paediatric population: a novel biomechanical approach. Clin Radiol. 73(10):839-54, 2018
                                          3. Lopez AJ et al: Anatomy and biomechanics of the craniovertebral junction. Neurosurg Focus. 38(4):E2, 2015
                                          4. Benglis D et al: Neurologic findings of craniovertebral junction disease. Neurosurgery. 66(3 Suppl):13-21, 2010
                                          5. Hankinson TC et al: Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. 66(3 Suppl):32-8, 2010
                                          6. Menezes AH et al: Remnants of occipital vertebrae: proatlas segmentation abnormalities. Neurosurgery. 64(5):945-53; discussion 954, 2009
                                          7. Kumar R et al: Craniovertebral junction anomaly with atlas assimilation and reducible atlantoaxial dislocation: a rare constellation of bony abnormalities. Pediatr Neurosurg. 44(5):402-5, 2008
                                          8. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                                          9. Smoker WR et al: Imaging the craniocervical junction. Childs Nerv Syst. 24(10):1123-45, 2008
                                          10. Gholve PA et al: Occipitalization of the atlas in children. Morphologic classification, associations, and clinical relevance. J Bone Joint Surg Am. 89(3):571-8, 2007