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Split Cord Malformation
Usha D. Nagaraj, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Diastematomyelia, diplomyelia
            • Definitions

              • Split cord malformation (SCM): Sagittal division of spinal cord into 2 hemicords
              • 2 main types
                • Type I SCM: 2 hemicords within separate dural tubes separated by rigid median septum
                • Type II SCM: 2 hemicords in single dural tube separated by nonrigid fibrous median septum
              • Terms diastematomyelia & diplomyelia have fallen out of favor as they can be difficult to distinguish & are frequently used interchangeably
                • Diastematomyelia: Sagittal division of spinal cord into 2 hemicords, each containing central canal, 1 dorsal horn, & 1 ventral horn
                • Diplomyelia (rare): Duplication of spinal cord with each cord containing central canal, 2 dorsal horns, & 2 ventral horns

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • 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

                                          • 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. Erşahin Y: Split cord malformation types I and II: a personal series of 131 patients. Childs Nerv Syst. 29(9):1515-26, 2013
                                            3. Barkovich A et al: Pediatric neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 857-922, 2012
                                            4. Mahapatra AK: Split cord malformation - A study of 300 cases at AIIMS 1990-2006. J Pediatr Neurosci. 6(Suppl 1):S41-5, 2011
                                            5. Muroi A et al: Split medulla in association with multiple closed neural tube defects. Childs Nerv Syst. Epub ahead of print, 2010
                                            6. 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
                                            7. 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
                                            8. 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
                                            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: Comparative study of complex spina bifida and split cord malformation. Indian J Pediatr. 72(2):109-15, 2005
                                            12. Mahapatra AK et al: Split cord malformations: a clinical study of 254 patients and a proposal for a new clinical-imaging classification. J Neurosurg. 103(6 Suppl):531-6, 2005
                                            13. Proctor MR et al: Long-term outcome for patients with split cord malformation. Neurosurg Focus. 10(1):e5, 2001
                                            14. Balci S et al: Diastematomyelia in two sisters. Am J Med Genet. 86(2):180-2, 1999
                                            15. Ersahin Y et al: Split spinal cord malformations in children. J Neurosurg. 88(1):57-65, 1998
                                            16. Kaffenberger DA et al: Meningocele manque: radiologic findings with clinical correlation. AJNR Am J Neuroradiol. 13(4):1083-8, 1992
                                            17. 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
                                            18. 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
                                            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

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Diastematomyelia, diplomyelia
                                                      • Definitions

                                                        • Split cord malformation (SCM): Sagittal division of spinal cord into 2 hemicords
                                                        • 2 main types
                                                          • Type I SCM: 2 hemicords within separate dural tubes separated by rigid median septum
                                                          • Type II SCM: 2 hemicords in single dural tube separated by nonrigid fibrous median septum
                                                        • Terms diastematomyelia & diplomyelia have fallen out of favor as they can be difficult to distinguish & are frequently used interchangeably
                                                          • Diastematomyelia: Sagittal division of spinal cord into 2 hemicords, each containing central canal, 1 dorsal horn, & 1 ventral horn
                                                          • Diplomyelia (rare): Duplication of spinal cord with each cord containing central canal, 2 dorsal horns, & 2 ventral horns

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • 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

                                                                                    • 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. Erşahin Y: Split cord malformation types I and II: a personal series of 131 patients. Childs Nerv Syst. 29(9):1515-26, 2013
                                                                                      3. Barkovich A et al: Pediatric neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 857-922, 2012
                                                                                      4. Mahapatra AK: Split cord malformation - A study of 300 cases at AIIMS 1990-2006. J Pediatr Neurosci. 6(Suppl 1):S41-5, 2011
                                                                                      5. Muroi A et al: Split medulla in association with multiple closed neural tube defects. Childs Nerv Syst. Epub ahead of print, 2010
                                                                                      6. 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
                                                                                      7. 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
                                                                                      8. 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
                                                                                      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: Comparative study of complex spina bifida and split cord malformation. Indian J Pediatr. 72(2):109-15, 2005
                                                                                      12. Mahapatra AK et al: Split cord malformations: a clinical study of 254 patients and a proposal for a new clinical-imaging classification. J Neurosurg. 103(6 Suppl):531-6, 2005
                                                                                      13. Proctor MR et al: Long-term outcome for patients with split cord malformation. Neurosurg Focus. 10(1):e5, 2001
                                                                                      14. Balci S et al: Diastematomyelia in two sisters. Am J Med Genet. 86(2):180-2, 1999
                                                                                      15. Ersahin Y et al: Split spinal cord malformations in children. J Neurosurg. 88(1):57-65, 1998
                                                                                      16. Kaffenberger DA et al: Meningocele manque: radiologic findings with clinical correlation. AJNR Am J Neuroradiol. 13(4):1083-8, 1992
                                                                                      17. 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
                                                                                      18. 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