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Syringomyelia
Blaise V. Jones, MD
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KEY FACTS

  • 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 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. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                          2. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                          3. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                          4. 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
                                          5. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                          6. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                          7. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                          8. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                          9. 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
                                          10. 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
                                          11. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                          12. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                          13. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                          14. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                          15. 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

                                          • 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 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. Rodriguez A et al: Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 16(4):452-7, 2015
                                                                                  2. Timpone VM et al: MRI of a syrinx: is contrast material always necessary? AJR Am J Roentgenol. 204(5):1082-5, 2015
                                                                                  3. McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014
                                                                                  4. 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
                                                                                  5. Magge SN et al: Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 7(1):30-6, 2011
                                                                                  6. Agarwal A et al: Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Pediatr Radiol. 40 Suppl 1:S156, 2010
                                                                                  7. Durnford AJ et al: Spontaneous regression of a terminal myelocystocele and associated syringomyelia: a case report. Childs Nerv Syst. 26(3):403-6, 2010
                                                                                  8. Heiss JD et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine. 13(6):729-42, 2010
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. Aitken LA et al: Chiari type I malformation in a pediatric population. Pediatr Neurol. 40(6):449-54, 2009
                                                                                  12. Nakamura M et al: Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech. 22(5):372-5, 2009
                                                                                  13. Ravaglia S et al: Presyrinx in children with Chiari malformations. Neurology. 72(22):1966-7, 2009
                                                                                  14. Akhtar OH et al: Syringomyelia-associated scoliosis with and without the Chiari I malformation. J Am Acad Orthop Surg. 16(7):407-17, 2008
                                                                                  15. Goh S et al: Presyrinx in children with Chiari malformations. Neurology. 71(5):351-6, 2008