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Craniovertebral Junction Variants
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Craniovertebral junction (CVJ), craniocerebral junction (CCJ)
              • Synonyms

                • CVJ anomalies, CCJ variants or anomalies
              • Definitions

                • Anatomic variations of skull base-upper cervical spine articulation

              IMAGING

              • General Features

                • Radiographic Findings

                  • Fluoroscopic Findings

                    • CT Findings

                      • MR Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Basaran R et al: Morphometric analysis of posterior fossa and craniovertebral junction in subtypes of Chiari malformation. Clin Neurol Neurosurg. 169:1-11, 2018
                                                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. Menezes AH: Nosographic identification and management of pediatric craniovertebral junction anomalies: evolution of concepts and modalities of treatment. Adv Tech Stand Neurosurg. 40:3-18, 2014
                                                4. Natung T et al: Symmetrical chorioretinal colobomata with craniovertebral junction anomalies in CHARGE syndrome - a case report with review of literature. J Clin Imaging Sci. 4:5, 2014
                                                5. Shetty SR et al: Neurenteric cyst at the craniovertebral junction: a report of two cases. Asian J Neurosurg. 8(4):188-91, 2013
                                                6. Tubbs RS et al: Hypoplastic occipital condyle and third occipital condyle: review of their dysembryology. Clin Anat. 26(8):928-32, 2013
                                                7. Menezes AH: Craniocervical fusions in children. J Neurosurg Pediatr. 9(6):573-85, 2012
                                                8. Muhleman M et al: The proatlas: a comprehensive review with clinical implications. Childs Nerv Syst. 28(3):349-56, 2012
                                                9. Hankinson TC et al: Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. 66(3 Suppl):32-8, 2010
                                                10. Smith JS et al: Basilar invagination. Neurosurgery. 66(3 Suppl):39-47, 2010
                                                11. Menezes AH et al: Remnants of occipital vertebrae: proatlas segmentation abnormalities. Neurosurgery. 64(5):945-53; discussion 954, 2009
                                                12. Menezes AH et al: Anatomy and biomechanics of normal craniovertebral junction (a) and biomechanics of stabilization (b). Childs Nerv Syst. 24(10):1091-100, 2008
                                                13. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                                                14. Smoker WR et al: Imaging the craniocervical junction. Childs Nerv Syst. 24(10):1123-45, 2008
                                                15. Naderi S et al: Anatomical and computed tomographic analysis of C1 vertebra. Clin Neurol Neurosurg. 105(4):245-8, 2003
                                                16. Perez-Vallina JR et al: Congenital anomaly of craniovertebral junction: atlas-dens fusion with C1 anterior arch cleft. J Spinal Disord Tech. 15(1):84-7, 2002
                                                17. Kim FM: Developmental anomalies of the craniocervical junction and cervical spine. Magn Reson Imaging Clin N Am. 8(3):651-74, 2000
                                                18. Smoker WR: MR imaging of the craniovertebral junction. Magn Reson Imaging Clin N Am. 8(3):635-50, 2000
                                                19. Taitz C: Bony observations of some morphological variations and anomalies of the craniovertebral region. Clin Anat. 13(5):354-60, 2000
                                                20. Iwata A et al: Foramen magnum syndrome caused by atlanto-occipital assimilation. J Neurol Sci. 154(2):229-31, 1998
                                                21. Menezes AH: Craniovertebral junction anomalies: diagnosis and management. Semin Pediatr Neurol. 4(3):209-23, 1997
                                                22. Menezes AH: Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg. 23(5):260-9, 1995
                                                23. Erbengi A et al: Congenital malformations of the craniovertebral junction: classification and surgical treatment. Acta Neurochir (Wien). 127(3-4):180-5, 1994
                                                24. Smoker WR: Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies. Radiographics. 14(2):255-77, 1994
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Craniovertebral junction (CVJ), craniocerebral junction (CCJ)
                                                            • Synonyms

                                                              • CVJ anomalies, CCJ variants or anomalies
                                                            • Definitions

                                                              • Anatomic variations of skull base-upper cervical spine articulation

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • Fluoroscopic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Basaran R et al: Morphometric analysis of posterior fossa and craniovertebral junction in subtypes of Chiari malformation. Clin Neurol Neurosurg. 169:1-11, 2018
                                                                                              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. Menezes AH: Nosographic identification and management of pediatric craniovertebral junction anomalies: evolution of concepts and modalities of treatment. Adv Tech Stand Neurosurg. 40:3-18, 2014
                                                                                              4. Natung T et al: Symmetrical chorioretinal colobomata with craniovertebral junction anomalies in CHARGE syndrome - a case report with review of literature. J Clin Imaging Sci. 4:5, 2014
                                                                                              5. Shetty SR et al: Neurenteric cyst at the craniovertebral junction: a report of two cases. Asian J Neurosurg. 8(4):188-91, 2013
                                                                                              6. Tubbs RS et al: Hypoplastic occipital condyle and third occipital condyle: review of their dysembryology. Clin Anat. 26(8):928-32, 2013
                                                                                              7. Menezes AH: Craniocervical fusions in children. J Neurosurg Pediatr. 9(6):573-85, 2012
                                                                                              8. Muhleman M et al: The proatlas: a comprehensive review with clinical implications. Childs Nerv Syst. 28(3):349-56, 2012
                                                                                              9. Hankinson TC et al: Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. 66(3 Suppl):32-8, 2010
                                                                                              10. Smith JS et al: Basilar invagination. Neurosurgery. 66(3 Suppl):39-47, 2010
                                                                                              11. Menezes AH et al: Remnants of occipital vertebrae: proatlas segmentation abnormalities. Neurosurgery. 64(5):945-53; discussion 954, 2009
                                                                                              12. Menezes AH et al: Anatomy and biomechanics of normal craniovertebral junction (a) and biomechanics of stabilization (b). Childs Nerv Syst. 24(10):1091-100, 2008
                                                                                              13. Menezes AH: Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 24(10):1109-22, 2008
                                                                                              14. Smoker WR et al: Imaging the craniocervical junction. Childs Nerv Syst. 24(10):1123-45, 2008
                                                                                              15. Naderi S et al: Anatomical and computed tomographic analysis of C1 vertebra. Clin Neurol Neurosurg. 105(4):245-8, 2003
                                                                                              16. Perez-Vallina JR et al: Congenital anomaly of craniovertebral junction: atlas-dens fusion with C1 anterior arch cleft. J Spinal Disord Tech. 15(1):84-7, 2002
                                                                                              17. Kim FM: Developmental anomalies of the craniocervical junction and cervical spine. Magn Reson Imaging Clin N Am. 8(3):651-74, 2000
                                                                                              18. Smoker WR: MR imaging of the craniovertebral junction. Magn Reson Imaging Clin N Am. 8(3):635-50, 2000
                                                                                              19. Taitz C: Bony observations of some morphological variations and anomalies of the craniovertebral region. Clin Anat. 13(5):354-60, 2000
                                                                                              20. Iwata A et al: Foramen magnum syndrome caused by atlanto-occipital assimilation. J Neurol Sci. 154(2):229-31, 1998
                                                                                              21. Menezes AH: Craniovertebral junction anomalies: diagnosis and management. Semin Pediatr Neurol. 4(3):209-23, 1997
                                                                                              22. Menezes AH: Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg. 23(5):260-9, 1995
                                                                                              23. Erbengi A et al: Congenital malformations of the craniovertebral junction: classification and surgical treatment. Acta Neurochir (Wien). 127(3-4):180-5, 1994
                                                                                              24. Smoker WR: Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies. Radiographics. 14(2):255-77, 1994