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Diastematomyelia
Kevin R. Moore, MD
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19
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Split cord malformation (SCM), "diastem"
              • Definitions

                • Sagittal division of spinal cord into 2 hemicords, each with 1 central canal, dorsal horn, and ventral horn

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Nonvascular Interventions

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Staging, Grading, & Classification

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Consider

                                                • Image Interpretation Pearls

                                                  Selected References

                                                  1. Alzhrani GA et al: Multi-level Split Cord Malformation: Do We Need a New Classification? J Clin Imaging Sci. 4:32, 2014
                                                  2. Burnei G et al: L3-L5 Teratological Spondylolysis with Diastematomyelia and L4 Radicular Syndrome followed by Spondyloschisis without Myelomeningocele due to Somato-arcuate Shifting. Spine J. ePub, 2014
                                                  3. Muroi A et al: Split medulla in association with multiple closed neural tube defects. Childs Nerv Syst. Epub ahead of print, 2010
                                                  4. Ayvaz M et al: Is it necessary to operate all split cord malformations before corrective surgery for patients with congenital spinal deformities? Spine (Phila Pa 1976). 34(22):2413-8, 2009
                                                  5. Liu W et al: Characteristics of osseous septum of split cord malformation in patients presenting with scoliosis: a retrospective study of 48 cases. Pediatr Neurosurg. 2009;45(5):350-3. Epub 2009 Nov 11. Erratum in: Pediatr Neurosurg. 45(5):406, 2009
                                                  6. Rowley VB et al: Lumbar split cord malformation with lateral hemimyelomeningocele and associated Chiari II malformation and other visceral and osseous anomalies: a case report. J Comput Assist Tomogr. 33(6):923-6, 2009
                                                  7. Ozturk E et al: Split-cord malformation and accompanying anomalies. J Neuroradiol. 35(3):150-6, 2008
                                                  8. Akiyama K et al: A case of split cord malformation associated with myeloschisis. Childs Nerv Syst. 23(5):577-80, 2007
                                                  9. Ansari S et al: Split cord malformation associated with myelomeningocele. J Neurosurg. 107(4 Suppl):281-5, 2007
                                                  10. Jankowitz BT et al: Cutaneous manifestations of split cord malformations. Report of three cases. J Neurosurg. 107(3 Suppl):240-3, 2007
                                                  11. Kumar R et al: Unusual split cord with neurenteric cyst and cerebellar heterotopia over spinal cord. Childs Nerv Syst. 23(2):243-7, 2007
                                                  12. Gupta DK et al: Proposal for a new clinicoradiological classification of type I split-cord malformations: a prospective study of 25 cases. Pediatr Neurosurg. 42(6):341-6, 2006
                                                  13. Moriya J et al: An unusual case of split cord malformation. AJNR Am J Neuroradiol. 27(7):1562-4, 2006
                                                  14. Balci S et al: Cervical diastematomyelia in cervico-oculo-acoustic (Wildervanck) syndrome: MRI findings. Clin Dysmorphol. 11(2):125-8, 2002
                                                  15. Proctor MR et al: Long-term outcome for patients with split cord malformation. Neurosurg Focus. 10(1):e5, 2001
                                                  16. Tortori-Donati P et al: Magnetic resonance imaging of spinal dysraphism. Top Magn Reson Imaging. 12(6):375-409, 2001
                                                  17. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia: Lippincott-Raven. 655-65, 2000
                                                  18. Balci S et al: Diastematomyelia in two sisters. Am J Med Genet. 86(2):180-2, 1999
                                                  19. Ersahin Y et al: Split spinal cord malformations in children. J Neurosurg. 88(1):57-65, 1998
                                                  20. Frymoyer et al: The Adult Spine. Vol 2. 2nd ed. Philadelphia: Lippincott-Raven. 1923-26, 1997
                                                  21. Miller A et al: Evaluation and treatment of diastematomyelia. J Bone Joint Surg Am. 75(9):1308-17, 1993
                                                  22. Kaffenberger DA et al: Meningocele manque: radiologic findings with clinical correlation. AJNR Am J Neuroradiol. 13(4):1083-8, 1992
                                                  23. Pang D et al: Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 31(3):451-80, 1992
                                                  24. Pang D: Split cord malformation: Part II: Clinical syndrome. Neurosurgery. 31(3):481-500, 1992
                                                  25. Naidich TP et al: Diastematomyelia: hemicord and meningeal sheaths; single and double arachnoid and dural tubes. AJNR Am J Neuroradiol. 4(3):633-6, 1983
                                                  26. Harwood-Nash DC et al: Diastematomyelia in 172 children: the impact of modern neuroradiology. Pediatr Neurosurg. 16(4-5):247-51, 1990-91
                                                  27. Russell NA et al: Diastematomyelia in adults. A review. Pediatr Neurosurg. 16(4-5):252-7, 1990-91
                                                  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

                                                              • Synonyms

                                                                • Split cord malformation (SCM), "diastem"
                                                              • Definitions

                                                                • Sagittal division of spinal cord into 2 hemicords, each with 1 central canal, dorsal horn, and ventral horn

                                                              IMAGING

                                                              • General Features

                                                                • Radiographic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Ultrasonographic Findings

                                                                        • Nonvascular Interventions

                                                                          • Imaging Recommendations

                                                                            DIFFERENTIAL DIAGNOSIS

                                                                              PATHOLOGY

                                                                              • General Features

                                                                                • Staging, Grading, & Classification

                                                                                  • Gross Pathologic & Surgical Features

                                                                                    • Microscopic Features

                                                                                      CLINICAL ISSUES

                                                                                      • Presentation

                                                                                        • Demographics

                                                                                          • Natural History & Prognosis

                                                                                            • Treatment

                                                                                              DIAGNOSTIC CHECKLIST

                                                                                              • Consider

                                                                                                • Image Interpretation Pearls

                                                                                                  Selected References

                                                                                                  1. Alzhrani GA et al: Multi-level Split Cord Malformation: Do We Need a New Classification? J Clin Imaging Sci. 4:32, 2014
                                                                                                  2. Burnei G et al: L3-L5 Teratological Spondylolysis with Diastematomyelia and L4 Radicular Syndrome followed by Spondyloschisis without Myelomeningocele due to Somato-arcuate Shifting. Spine J. ePub, 2014
                                                                                                  3. Muroi A et al: Split medulla in association with multiple closed neural tube defects. Childs Nerv Syst. Epub ahead of print, 2010
                                                                                                  4. Ayvaz M et al: Is it necessary to operate all split cord malformations before corrective surgery for patients with congenital spinal deformities? Spine (Phila Pa 1976). 34(22):2413-8, 2009
                                                                                                  5. Liu W et al: Characteristics of osseous septum of split cord malformation in patients presenting with scoliosis: a retrospective study of 48 cases. Pediatr Neurosurg. 2009;45(5):350-3. Epub 2009 Nov 11. Erratum in: Pediatr Neurosurg. 45(5):406, 2009
                                                                                                  6. Rowley VB et al: Lumbar split cord malformation with lateral hemimyelomeningocele and associated Chiari II malformation and other visceral and osseous anomalies: a case report. J Comput Assist Tomogr. 33(6):923-6, 2009
                                                                                                  7. Ozturk E et al: Split-cord malformation and accompanying anomalies. J Neuroradiol. 35(3):150-6, 2008
                                                                                                  8. Akiyama K et al: A case of split cord malformation associated with myeloschisis. Childs Nerv Syst. 23(5):577-80, 2007
                                                                                                  9. Ansari S et al: Split cord malformation associated with myelomeningocele. J Neurosurg. 107(4 Suppl):281-5, 2007
                                                                                                  10. Jankowitz BT et al: Cutaneous manifestations of split cord malformations. Report of three cases. J Neurosurg. 107(3 Suppl):240-3, 2007
                                                                                                  11. Kumar R et al: Unusual split cord with neurenteric cyst and cerebellar heterotopia over spinal cord. Childs Nerv Syst. 23(2):243-7, 2007
                                                                                                  12. Gupta DK et al: Proposal for a new clinicoradiological classification of type I split-cord malformations: a prospective study of 25 cases. Pediatr Neurosurg. 42(6):341-6, 2006
                                                                                                  13. Moriya J et al: An unusual case of split cord malformation. AJNR Am J Neuroradiol. 27(7):1562-4, 2006
                                                                                                  14. Balci S et al: Cervical diastematomyelia in cervico-oculo-acoustic (Wildervanck) syndrome: MRI findings. Clin Dysmorphol. 11(2):125-8, 2002
                                                                                                  15. Proctor MR et al: Long-term outcome for patients with split cord malformation. Neurosurg Focus. 10(1):e5, 2001
                                                                                                  16. Tortori-Donati P et al: Magnetic resonance imaging of spinal dysraphism. Top Magn Reson Imaging. 12(6):375-409, 2001
                                                                                                  17. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia: Lippincott-Raven. 655-65, 2000
                                                                                                  18. Balci S et al: Diastematomyelia in two sisters. Am J Med Genet. 86(2):180-2, 1999
                                                                                                  19. Ersahin Y et al: Split spinal cord malformations in children. J Neurosurg. 88(1):57-65, 1998
                                                                                                  20. Frymoyer et al: The Adult Spine. Vol 2. 2nd ed. Philadelphia: Lippincott-Raven. 1923-26, 1997
                                                                                                  21. Miller A et al: Evaluation and treatment of diastematomyelia. J Bone Joint Surg Am. 75(9):1308-17, 1993
                                                                                                  22. Kaffenberger DA et al: Meningocele manque: radiologic findings with clinical correlation. AJNR Am J Neuroradiol. 13(4):1083-8, 1992
                                                                                                  23. Pang D et al: Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 31(3):451-80, 1992
                                                                                                  24. Pang D: Split cord malformation: Part II: Clinical syndrome. Neurosurgery. 31(3):481-500, 1992
                                                                                                  25. Naidich TP et al: Diastematomyelia: hemicord and meningeal sheaths; single and double arachnoid and dural tubes. AJNR Am J Neuroradiol. 4(3):633-6, 1983
                                                                                                  26. Harwood-Nash DC et al: Diastematomyelia in 172 children: the impact of modern neuroradiology. Pediatr Neurosurg. 16(4-5):247-51, 1990-91
                                                                                                  27. Russell NA et al: Diastematomyelia in adults. A review. Pediatr Neurosurg. 16(4-5):252-7, 1990-91