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Syringomyelia
Kevin R. Moore, MD; Blaise V. Jones, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            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
              • 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. Jia C et al: Comparison decompression by duraplasty or cerebellar tonsillectomy for Chiari malformation-I complicated with syringomyelia. Clin Neurol Neurosurg. 176:1-7, 2018
                                              2. Menezes AH et al: Syringobulbia in pediatric patients with Chiari malformation type I. J Neurosurg Pediatr. 22(1):52-60, 2018
                                              3. Singhal A et al: International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions. Childs Nerv Syst. 34(6):1177-82, 2018
                                              4. Vinje V et al: Fluid dynamics in syringomyelia cavities: effects of heart rate, CSF velocity, CSF velocity waveform and craniovertebral decompression. Neuroradiol J. 31(5):482-89, 2018
                                              5. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                              6. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                              7. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                              8. 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
                                              9. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                              10. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                              11. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                              12. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                              13. 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
                                              14. 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
                                              15. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                              16. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                              17. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                              18. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                              19. 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

                                                      • Diagnostic Checklist

                                                        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
                                                          • 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. Jia C et al: Comparison decompression by duraplasty or cerebellar tonsillectomy for Chiari malformation-I complicated with syringomyelia. Clin Neurol Neurosurg. 176:1-7, 2018
                                                                                          2. Menezes AH et al: Syringobulbia in pediatric patients with Chiari malformation type I. J Neurosurg Pediatr. 22(1):52-60, 2018
                                                                                          3. Singhal A et al: International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions. Childs Nerv Syst. 34(6):1177-82, 2018
                                                                                          4. Vinje V et al: Fluid dynamics in syringomyelia cavities: effects of heart rate, CSF velocity, CSF velocity waveform and craniovertebral decompression. Neuroradiol J. 31(5):482-89, 2018
                                                                                          5. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                                                                          6. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                                                                          7. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                                                                          8. 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
                                                                                          9. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                                                                          10. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                                                                          11. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                                                                          12. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                                                                          13. 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
                                                                                          14. 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
                                                                                          15. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                                                                          16. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                                                                          17. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                                                          18. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                                                          19. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008