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Syrinx, Craniovertebral Junction
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Hydromyelia: Cystic central canal dilatation
                • Syringomyelia: Cystic spinal cord cavity not contiguous with central cord canal
                  • Syringobulbia: Brainstem syrinx extension
                  • Syringocephaly: Brain/cerebral peduncle syrinx extension
                • Syringohydromyelia: Features of both syringomyelia and hydromyelia
                • "Presyrinx state": Reversible spinal cord edema produced by alterations in CSF flow dynamics

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Nonvascular Interventions

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Massimi L et al: Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications. Neurosurg Rev. 35(3):321-9, 2012
                                                2. Muroi A et al: Giant syringobulbia associated with cerebellopontine angle arachnoid cyst and hydrocephalus. J Neurosurg Pediatr. 8(1):30-4, 2011
                                                3. Aghakhani N et al: Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery. 64(2):308-15; discussion 315, 2009
                                                4. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                5. Tubbs RS et al: Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia. Childs Nerv Syst. 25(6):689-92, 2009
                                                6. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                7. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008
                                                8. Park CH et al: Evaluation of intrasyrinx fluid motion by spatial modulation of magnetization-magnetic resonance imaging in syringomyelia with long-term follow-up: a predictor of postoperative prognosis? J Comput Assist Tomogr. 32(1):135-40, 2008
                                                9. Sung WS et al: Spontaneous regression of syringomyelia--review of the current aetiological theories and implications for surgery. J Clin Neurosci. 15(10):1185-8, 2008
                                                10. Ravaglia S et al: Pathogenetic role of myelitis for syringomyelia. Clin Neurol Neurosurg. 109(6):541-6, 2007
                                                11. Yeom JS et al: Scoliosis associated with syringomyelia: analysis of MRI and curve progression. Eur Spine J. 16(10):1629-35, 2007
                                                12. Balédent O et al: Value of phase contrast magnetic resonance imaging for investigation of cerebral hydrodynamics. J Neuroradiol. 33(5):292-303, 2006
                                                13. Aryan HE et al: Syringocephaly. J Clin Neurosci. 11(4):421-3, 2004
                                                14. Piatt JH Jr: Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg. 100(2 Suppl):101-9, 2004
                                                15. Brodbelt AR et al: Post-traumatic syringomyelia: a review. J Clin Neurosci. 10(4):401-8, 2003
                                                16. Kyoshima K et al: Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 53(3):762-8; discussion 768-9, 2003
                                                17. Ozerdemoglu RA et al: Scoliosis associated with syringomyelia: clinical and radiologic correlation. Spine. 28(13):1410-7, 2003
                                                18. Klekamp J: The pathophysiology of syringomyelia - historical overview and current concept. Acta Neurochir (Wien). 144(7):649-64, 2002
                                                19. Klekamp J et al: Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature. Neurosurgery. 48(3):664-7, 2001
                                                20. Vinas FC et al: Spontaneous resolution of a syrinx. J Clin Neurosci. 8(2):170-2, 2001
                                                21. Brugières P et al: CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol. 21(10):1785-92, 2000
                                                22. Fischbein NJ et al: The "presyrinx" state: is there a reversible myelopathic condition that may precede syringomyelia? Neurosurg Focus. 8(3):E4, 2000
                                                23. Fischbein NJ et al: The "presyrinx" state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol. 20(1):7-20, 1999
                                                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

                                                            • Definitions

                                                              • Hydromyelia: Cystic central canal dilatation
                                                              • Syringomyelia: Cystic spinal cord cavity not contiguous with central cord canal
                                                                • Syringobulbia: Brainstem syrinx extension
                                                                • Syringocephaly: Brain/cerebral peduncle syrinx extension
                                                              • Syringohydromyelia: Features of both syringomyelia and hydromyelia
                                                              • "Presyrinx state": Reversible spinal cord edema produced by alterations in CSF flow dynamics

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic Findings

                                                                      • Nonvascular Interventions

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Massimi L et al: Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications. Neurosurg Rev. 35(3):321-9, 2012
                                                                                              2. Muroi A et al: Giant syringobulbia associated with cerebellopontine angle arachnoid cyst and hydrocephalus. J Neurosurg Pediatr. 8(1):30-4, 2011
                                                                                              3. Aghakhani N et al: Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery. 64(2):308-15; discussion 315, 2009
                                                                                              4. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                                                              5. Tubbs RS et al: Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia. Childs Nerv Syst. 25(6):689-92, 2009
                                                                                              6. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                                                              7. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008
                                                                                              8. Park CH et al: Evaluation of intrasyrinx fluid motion by spatial modulation of magnetization-magnetic resonance imaging in syringomyelia with long-term follow-up: a predictor of postoperative prognosis? J Comput Assist Tomogr. 32(1):135-40, 2008
                                                                                              9. Sung WS et al: Spontaneous regression of syringomyelia--review of the current aetiological theories and implications for surgery. J Clin Neurosci. 15(10):1185-8, 2008
                                                                                              10. Ravaglia S et al: Pathogenetic role of myelitis for syringomyelia. Clin Neurol Neurosurg. 109(6):541-6, 2007
                                                                                              11. Yeom JS et al: Scoliosis associated with syringomyelia: analysis of MRI and curve progression. Eur Spine J. 16(10):1629-35, 2007
                                                                                              12. Balédent O et al: Value of phase contrast magnetic resonance imaging for investigation of cerebral hydrodynamics. J Neuroradiol. 33(5):292-303, 2006
                                                                                              13. Aryan HE et al: Syringocephaly. J Clin Neurosci. 11(4):421-3, 2004
                                                                                              14. Piatt JH Jr: Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg. 100(2 Suppl):101-9, 2004
                                                                                              15. Brodbelt AR et al: Post-traumatic syringomyelia: a review. J Clin Neurosci. 10(4):401-8, 2003
                                                                                              16. Kyoshima K et al: Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 53(3):762-8; discussion 768-9, 2003
                                                                                              17. Ozerdemoglu RA et al: Scoliosis associated with syringomyelia: clinical and radiologic correlation. Spine. 28(13):1410-7, 2003
                                                                                              18. Klekamp J: The pathophysiology of syringomyelia - historical overview and current concept. Acta Neurochir (Wien). 144(7):649-64, 2002
                                                                                              19. Klekamp J et al: Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature. Neurosurgery. 48(3):664-7, 2001
                                                                                              20. Vinas FC et al: Spontaneous resolution of a syrinx. J Clin Neurosci. 8(2):170-2, 2001
                                                                                              21. Brugières P et al: CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol. 21(10):1785-92, 2000
                                                                                              22. Fischbein NJ et al: The "presyrinx" state: is there a reversible myelopathic condition that may precede syringomyelia? Neurosurg Focus. 8(3):E4, 2000
                                                                                              23. Fischbein NJ et al: The "presyrinx" state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol. 20(1):7-20, 1999