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Syringomyelia
Blaise V. Jones, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Hydromyelia, syringohydromyelia, syrinx
          • Definitions

            • Hydromyelia: Cystic dilation of spinal cord central canal
            • Syringomyelia: Cystic spinal cord cavity not contiguous with central canal
            • Syringohydromyelia: Features of both syringomyelia & hydromyelia
              • Distinction between syringomyelia & hydromyelia is often not clinically relevant
            • Syringobulbia: Extension of syrinx/cavity into brainstem
            • Syringocephaly: Brain/cerebral peduncle syrinx extension
            • "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. Kurzbuch AR et al: Iatrogenic syringomyelia postcranial surgery in pediatric patients: systematic review and illustrative case. Childs Nerv Syst. 37(9):2879-90, 2021
                                            2. Shenoy VS et al: Syringomyelia. StatPearls, 2020
                                            3. Heiss JD et al: Origin of syrinx fluid in syringomyelia: a physiological study. Neurosurgery. 84(2):457-68, 2019
                                            4. Holly LT et al: Chiari malformation and syringomyelia. J Neurosurg Spine. 31(5):619-28, 2019
                                            5. Jones BV: Cord cystic cavities: syringomyelia and prominent central canal. Semin Ultrasound CT MR. 38(2):98-104, 2017
                                            6. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                            7. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                            8. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                            9. Joseph RN et al: Management of isolated syringomyelia in the paediatric population--a review of imaging and follow-up in a single centre. Br J Neurosurg. 27(5):683-6, 2013
                                            10. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                            11. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                            12. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                            13. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                            14. Roser F et al: Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies. Acta Neurochir (Wien). 152(2):213-9; discussion 219, 2010
                                            15. 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
                                            16. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                            17. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                            18. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                            19. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                            20. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Hydromyelia, syringohydromyelia, syrinx
                                                    • Definitions

                                                      • Hydromyelia: Cystic dilation of spinal cord central canal
                                                      • Syringomyelia: Cystic spinal cord cavity not contiguous with central canal
                                                      • Syringohydromyelia: Features of both syringomyelia & hydromyelia
                                                        • Distinction between syringomyelia & hydromyelia is often not clinically relevant
                                                      • Syringobulbia: Extension of syrinx/cavity into brainstem
                                                      • Syringocephaly: Brain/cerebral peduncle syrinx extension
                                                      • "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. Kurzbuch AR et al: Iatrogenic syringomyelia postcranial surgery in pediatric patients: systematic review and illustrative case. Childs Nerv Syst. 37(9):2879-90, 2021
                                                                                      2. Shenoy VS et al: Syringomyelia. StatPearls, 2020
                                                                                      3. Heiss JD et al: Origin of syrinx fluid in syringomyelia: a physiological study. Neurosurgery. 84(2):457-68, 2019
                                                                                      4. Holly LT et al: Chiari malformation and syringomyelia. J Neurosurg Spine. 31(5):619-28, 2019
                                                                                      5. Jones BV: Cord cystic cavities: syringomyelia and prominent central canal. Semin Ultrasound CT MR. 38(2):98-104, 2017
                                                                                      6. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                                                                      7. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                                                                      8. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                                                                      9. Joseph RN et al: Management of isolated syringomyelia in the paediatric population--a review of imaging and follow-up in a single centre. Br J Neurosurg. 27(5):683-6, 2013
                                                                                      10. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                                                                      11. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                                                                      12. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                                                                      13. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                                                                      14. Roser F et al: Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies. Acta Neurochir (Wien). 152(2):213-9; discussion 219, 2010
                                                                                      15. 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
                                                                                      16. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                                                                      17. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                                                                      18. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                                                      19. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                                                      20. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008