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Myelocystocele
Usha D. Nagaraj, MD
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KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Syringocele, lipomyelocystocele
            • Definitions

              • Closed spinal dysraphism characterized by syrinx associated with neural placode protruding outside of spinal canal through osseous dysraphic defect
              • Terminal and nonterminal subtypes
                • Terminal myelocystocele is spinal dysraphism involving caudal-most aspect of spinal cord
                • Nonterminal myelocystocele: 2 types
                  • Abortive nonterminal myelocystocele/myelocystocele manque ("limited dorsal myeloschisis")
                    • Appears as meningocele crossed by fibroneurovascular stable that extends from dorsal aspect of spinal cord to attach to dome of meningocele sac
                  • Complete form
                    • Hydromyelic cavity within sac is continuous with ependymal canal of spinal cord

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • 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. Muthukumar N: Terminal myelocystocele with holocord syringomyelia: short report. J Pediatr Neurosci. 8(2):171-2, 2013
                                            2. Barkovich AJ et al: Pediatric Neuroimaging. Fifth edition. Philadelphia, PA: Lippincott Williams & Wilkins. 888-90, 2012
                                            3. Pang D et al: Terminal myelocystocele: surgical observations and theory of embryogenesis. Neurosurgery. 70(6):1383-404; discussion 1404-5, 2012
                                            4. Tandon V et al: Terminal myelocystocele: a series of 30 cases and review of the literature. Pediatr Neurosurg. 48(4):229-35, 2012
                                            5. Hashiguchi K et al: Holocord hydrosyringomyelia with terminal myelocystocele revealed by constructive interference in steady-state MR imaging. Pediatr Neurosurg. 44(6):509-12, 2008
                                            6. Morioka T et al: Neurosurgical management of occult spinal dysraphism associated with OEIS complex. Childs Nerv Syst. 24(6):723-9, 2008
                                            7. Muthukumar N: Terminal and nonterminal myelocystoceles. J Neurosurg. 107(2 Suppl):87-97, 2007
                                            8. Rossi A et al: Spectrum of nonterminal myelocystoceles. Neurosurgery. 58(3):509-15; discussion 509-15, 2006
                                            9. Gupta DK et al: Terminal myelocystoceles: a series of 17 cases. J Neurosurg. 103(4 Suppl):344-52, 2005
                                            10. Tortori-Donati P et al: Pediatric Neuroradiology. Heidelburg, Germany. 1574-1576, 2005
                                            11. Rossi A et al: Imaging in spine and spinal cord malformations. Eur J Radiol. 50(2):177-200, 2004
                                            12. Tortori-Donati P et al: Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology. 42(7):471-91, 2000
                                            13. Byrd SE et al: Imaging of terminal myelocystoceles. J Natl Med Assoc. 88(8):510-6, 1996
                                            14. Steinbok P et al: Cervical meningoceles and myelocystoceles: a unifying hypothesis. Pediatr Neurosurg. 23(6):317-22, 1995
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Syringocele, lipomyelocystocele
                                                      • Definitions

                                                        • Closed spinal dysraphism characterized by syrinx associated with neural placode protruding outside of spinal canal through osseous dysraphic defect
                                                        • Terminal and nonterminal subtypes
                                                          • Terminal myelocystocele is spinal dysraphism involving caudal-most aspect of spinal cord
                                                          • Nonterminal myelocystocele: 2 types
                                                            • Abortive nonterminal myelocystocele/myelocystocele manque ("limited dorsal myeloschisis")
                                                              • Appears as meningocele crossed by fibroneurovascular stable that extends from dorsal aspect of spinal cord to attach to dome of meningocele sac
                                                            • Complete form
                                                              • Hydromyelic cavity within sac is continuous with ependymal canal of spinal cord

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • 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. Muthukumar N: Terminal myelocystocele with holocord syringomyelia: short report. J Pediatr Neurosci. 8(2):171-2, 2013
                                                                                      2. Barkovich AJ et al: Pediatric Neuroimaging. Fifth edition. Philadelphia, PA: Lippincott Williams & Wilkins. 888-90, 2012
                                                                                      3. Pang D et al: Terminal myelocystocele: surgical observations and theory of embryogenesis. Neurosurgery. 70(6):1383-404; discussion 1404-5, 2012
                                                                                      4. Tandon V et al: Terminal myelocystocele: a series of 30 cases and review of the literature. Pediatr Neurosurg. 48(4):229-35, 2012
                                                                                      5. Hashiguchi K et al: Holocord hydrosyringomyelia with terminal myelocystocele revealed by constructive interference in steady-state MR imaging. Pediatr Neurosurg. 44(6):509-12, 2008
                                                                                      6. Morioka T et al: Neurosurgical management of occult spinal dysraphism associated with OEIS complex. Childs Nerv Syst. 24(6):723-9, 2008
                                                                                      7. Muthukumar N: Terminal and nonterminal myelocystoceles. J Neurosurg. 107(2 Suppl):87-97, 2007
                                                                                      8. Rossi A et al: Spectrum of nonterminal myelocystoceles. Neurosurgery. 58(3):509-15; discussion 509-15, 2006
                                                                                      9. Gupta DK et al: Terminal myelocystoceles: a series of 17 cases. J Neurosurg. 103(4 Suppl):344-52, 2005
                                                                                      10. Tortori-Donati P et al: Pediatric Neuroradiology. Heidelburg, Germany. 1574-1576, 2005
                                                                                      11. Rossi A et al: Imaging in spine and spinal cord malformations. Eur J Radiol. 50(2):177-200, 2004
                                                                                      12. Tortori-Donati P et al: Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology. 42(7):471-91, 2000
                                                                                      13. Byrd SE et al: Imaging of terminal myelocystoceles. J Natl Med Assoc. 88(8):510-6, 1996
                                                                                      14. Steinbok P et al: Cervical meningoceles and myelocystoceles: a unifying hypothesis. Pediatr Neurosurg. 23(6):317-22, 1995