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Syringomyelia
Jeffrey S. Ross, 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

                          • Other Modality Findings

                            • 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. Governale LS: Resolution of Chiari-associated syringomyelia. Pediatr Neurol. 50(5):542-3, 2014
                                                  2. Kemp J et al: Holocord syringomyelia secondary to tethered spinal cord associated with anterior sacral meningocele and tailgut cyst: case report and review of literature. Childs Nerv Syst. 30(6):1141-6, 2014
                                                  3. Nagoshi N et al: Factors contributing to improvement of syringomyelia after foramen magnum decompression for Chiari type I malformation. J Orthop Sci. 19(3):418-23, 2014
                                                  4. Vandertop WP: Syringomyelia. Neuropediatrics. 45(1):3-9, 2014
                                                  5. Tsitouras V et al: Syringomyelia and tethered cord in children. Childs Nerv Syst. 29(9):1625-34, 2013
                                                  6. 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
                                                  7. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                                  8. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                  9. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                  10. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008
                                                  11. 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
                                                  12. 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
                                                  13. Porensky P et al: Nontraumatic cervicothoracic syrinx as a cause of progressive neurologic dysfunction. J Spinal Cord Med. 30(3):276-81, 2007
                                                  14. Ravaglia S et al: Pathogenetic role of myelitis for syringomyelia. Clin Neurol Neurosurg. 109(6):541-6, 2007
                                                  15. Aryan HE et al: Syringocephaly. J Clin Neurosci. 11(4):421-3, 2004
                                                  16. Piatt JH Jr: Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg. 100(2 Suppl):101-9, 2004
                                                  17. Brodbelt AR et al: Post-traumatic syringomyelia: a review. J Clin Neurosci. 10(4):401-8, 2003
                                                  18. 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
                                                  19. Ozerdemoglu RA et al: Scoliosis associated with syringomyelia: clinical and radiologic correlation. Spine. 28(13):1410-7, 2003
                                                  20. Holly LT et al: Slitlike syrinx cavities: a persistent central canal. J Neurosurg. 97(2 Suppl):161-5, 2002
                                                  21. Klekamp J et al: Syringomyelia associated with foramen magnum arachnoiditis. J Neurosurg. 97(3 Suppl):317-22, 2002
                                                  22. Klekamp J: The pathophysiology of syringomyelia - historical overview and current concept. Acta Neurochir (Wien). 144(7):649-64, 2002
                                                  23. 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
                                                  24. Vinas FC et al: Spontaneous resolution of a syrinx. J Clin Neurosci. 8(2):170-2, 2001
                                                  25. Brugières P et al: CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol. 21(10):1785-92, 2000
                                                  26. Fischbein NJ et al: The "presyrinx" state: is there a reversible myelopathic condition that may precede syringomyelia? Neurosurg Focus. 8(3):E4, 2000
                                                  27. Unsinn KM et al: US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases. Radiographics. 20(4):923-38, 2000
                                                  28. Fischbein NJ et al: The "presyrinx" state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol. 20(1):7-20, 1999
                                                  29. Levy LM: MR imaging of cerebrospinal fluid flow and spinal cord motion in neurologic disorders of the spine. Magn Reson Imaging Clin N Am. 7(3):573-87, 1999
                                                  30. Milhorat TH et al: Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. 41(5):1102-10, 1997
                                                  31. Milhorat TH et al: Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg. 82(5):802-12, 1995
                                                  32. Tanghe HL: Magnetic resonance imaging (MRI) in syringomyelia. Acta Neurochir (Wien). 134(1-2):93-9, 1995
                                                  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

                                                                          • Other Modality Findings

                                                                            • 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. Governale LS: Resolution of Chiari-associated syringomyelia. Pediatr Neurol. 50(5):542-3, 2014
                                                                                                  2. Kemp J et al: Holocord syringomyelia secondary to tethered spinal cord associated with anterior sacral meningocele and tailgut cyst: case report and review of literature. Childs Nerv Syst. 30(6):1141-6, 2014
                                                                                                  3. Nagoshi N et al: Factors contributing to improvement of syringomyelia after foramen magnum decompression for Chiari type I malformation. J Orthop Sci. 19(3):418-23, 2014
                                                                                                  4. Vandertop WP: Syringomyelia. Neuropediatrics. 45(1):3-9, 2014
                                                                                                  5. Tsitouras V et al: Syringomyelia and tethered cord in children. Childs Nerv Syst. 29(9):1625-34, 2013
                                                                                                  6. 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
                                                                                                  7. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                                                                                  8. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                                                                  9. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                                                                  10. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008
                                                                                                  11. 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
                                                                                                  12. 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
                                                                                                  13. Porensky P et al: Nontraumatic cervicothoracic syrinx as a cause of progressive neurologic dysfunction. J Spinal Cord Med. 30(3):276-81, 2007
                                                                                                  14. Ravaglia S et al: Pathogenetic role of myelitis for syringomyelia. Clin Neurol Neurosurg. 109(6):541-6, 2007
                                                                                                  15. Aryan HE et al: Syringocephaly. J Clin Neurosci. 11(4):421-3, 2004
                                                                                                  16. Piatt JH Jr: Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg. 100(2 Suppl):101-9, 2004
                                                                                                  17. Brodbelt AR et al: Post-traumatic syringomyelia: a review. J Clin Neurosci. 10(4):401-8, 2003
                                                                                                  18. 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
                                                                                                  19. Ozerdemoglu RA et al: Scoliosis associated with syringomyelia: clinical and radiologic correlation. Spine. 28(13):1410-7, 2003
                                                                                                  20. Holly LT et al: Slitlike syrinx cavities: a persistent central canal. J Neurosurg. 97(2 Suppl):161-5, 2002
                                                                                                  21. Klekamp J et al: Syringomyelia associated with foramen magnum arachnoiditis. J Neurosurg. 97(3 Suppl):317-22, 2002
                                                                                                  22. Klekamp J: The pathophysiology of syringomyelia - historical overview and current concept. Acta Neurochir (Wien). 144(7):649-64, 2002
                                                                                                  23. 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
                                                                                                  24. Vinas FC et al: Spontaneous resolution of a syrinx. J Clin Neurosci. 8(2):170-2, 2001
                                                                                                  25. Brugières P et al: CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol. 21(10):1785-92, 2000
                                                                                                  26. Fischbein NJ et al: The "presyrinx" state: is there a reversible myelopathic condition that may precede syringomyelia? Neurosurg Focus. 8(3):E4, 2000
                                                                                                  27. Unsinn KM et al: US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases. Radiographics. 20(4):923-38, 2000
                                                                                                  28. Fischbein NJ et al: The "presyrinx" state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol. 20(1):7-20, 1999
                                                                                                  29. Levy LM: MR imaging of cerebrospinal fluid flow and spinal cord motion in neurologic disorders of the spine. Magn Reson Imaging Clin N Am. 7(3):573-87, 1999
                                                                                                  30. Milhorat TH et al: Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. 41(5):1102-10, 1997
                                                                                                  31. Milhorat TH et al: Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg. 82(5):802-12, 1995
                                                                                                  32. Tanghe HL: Magnetic resonance imaging (MRI) in syringomyelia. Acta Neurochir (Wien). 134(1-2):93-9, 1995