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Myelomeningocele
Usha D. Nagaraj, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Meningomyelocele, myelocele/myeloschisis, open spinal dysraphism, spina bifida aperta
              • Definitions

                • Myelomeningocele (MMC): Open (lacking skin covering) spinal dysraphism in which neural placode (exposed flattened end of elongated spinal cord) protrudes as sac beyond skin surface dorsal to spinal canal
                • Myelocele (MC): Open spinal dysraphism with neural placode flush with skin surface or in spinal canal (no sac)
                • Some authors refer to all open spinal dysraphisms as MMC (as MMC & MC fall along same pathologic spectrum)

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Khalaveh F et al: Myelomeningocele-Chiari II malformation-neurological predictability based on fetal and postnatal magnetic resonance imaging. Prenat Diagn. 41(8):922-32, 2021
                                            2. Licci M et al: CSF shunting in myelomeningocele-related hydrocephalus and the role of prenatal imaging. Childs Nerv Syst. ePub, 2021
                                            3. Yuan W et al: Diffusion tensor imaging in children following prenatal myelomeningocele repair and its predictive value for the need and timing of subsequent CSF diversion surgery for hydrocephalus. J Neurosurg Pediatr. 1-9, 2021
                                            4. Nagaraj UD et al: Imaging of open spinal dysraphisms in the era of prenatal surgery. Pediatr Radiol. 50(13):1988-98, 2020
                                            5. Nagaraj UD et al: Myelomeningocele versus myelocele on fetal MR images: are there differences in brain findings? AJR Am J Roentgenol. 211(6):1376-80, 2018
                                            6. Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Wolters Kluwer. 979-82, 2019
                                            7. Nagaraj UD et al: Spinal imaging findings of open spinal dysraphisms on fetal and postnatal MRI. AJNR Am J Neuroradiol. 39(10):1947-52, 2018
                                            8. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                            9. Caldarelli M et al: Recurrent tethered cord: radiological investigation and management. Childs Nerv Syst. 29(9):1601-9, 2013
                                            10. Messing-Jünger M et al: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 29(9):1553-62, 2013
                                            11. Sweeney KJ et al: Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia. J Neurosurg Pediatr. 11(2):154-9, 2013
                                            12. Jernigan SC et al: Risk factors of sudden death in young adult patients with myelomeningocele. J Neurosurg Pediatr. 9(2):149-55, 2012
                                            13. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                            14. Chao TT et al: Fetal spine findings on MRI and associated outcomes in children with open neural tube defects. AJR Am J Roentgenol. 197(5):W956-61, 2011
                                            15. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                            16. Rufener SL et al: Congenital spine and spinal cord malformations--pictorial review. AJR Am J Roentgenol. 194(3 Suppl):S26-37, 2010
                                            17. Hirose S et al: Fetal surgery for myelomeningocele. Clin Perinatol. 36(2):431-8, xi, 2009
                                            18. Hirose S et al: Fetal surgery for myelomeningocele: panacea or peril? World J Surg. 27(1):87-94, 2003
                                            19. Johnson MP et al: Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol. 189(2):482-7, 2003
                                            20. Ertl-Wagner B et al: Fetal magnetic resonance imaging: indications, technique, anatomical considerations and a review of fetal abnormalities. Eur Radiol. 12(8):1931-40, 2002
                                            21. Rintoul NE et al: A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics. 109(3):409-13, 2002
                                            22. Trivedi J et al: Clinical and radiographic predictors of scoliosis in patients with myelomeningocele. J Bone Joint Surg Am. 84-A(8):1389-94, 2002
                                            23. Bowman RM et al: Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 34(3):114-20, 2001
                                            24. 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
                                            25. Unsinn KM et al: US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases. Radiographics. 20(4):923-38, 2000
                                            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

                                                          • Meningomyelocele, myelocele/myeloschisis, open spinal dysraphism, spina bifida aperta
                                                        • Definitions

                                                          • Myelomeningocele (MMC): Open (lacking skin covering) spinal dysraphism in which neural placode (exposed flattened end of elongated spinal cord) protrudes as sac beyond skin surface dorsal to spinal canal
                                                          • Myelocele (MC): Open spinal dysraphism with neural placode flush with skin surface or in spinal canal (no sac)
                                                          • Some authors refer to all open spinal dysraphisms as MMC (as MMC & MC fall along same pathologic spectrum)

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Gross Pathologic & Surgical Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Khalaveh F et al: Myelomeningocele-Chiari II malformation-neurological predictability based on fetal and postnatal magnetic resonance imaging. Prenat Diagn. 41(8):922-32, 2021
                                                                                      2. Licci M et al: CSF shunting in myelomeningocele-related hydrocephalus and the role of prenatal imaging. Childs Nerv Syst. ePub, 2021
                                                                                      3. Yuan W et al: Diffusion tensor imaging in children following prenatal myelomeningocele repair and its predictive value for the need and timing of subsequent CSF diversion surgery for hydrocephalus. J Neurosurg Pediatr. 1-9, 2021
                                                                                      4. Nagaraj UD et al: Imaging of open spinal dysraphisms in the era of prenatal surgery. Pediatr Radiol. 50(13):1988-98, 2020
                                                                                      5. Nagaraj UD et al: Myelomeningocele versus myelocele on fetal MR images: are there differences in brain findings? AJR Am J Roentgenol. 211(6):1376-80, 2018
                                                                                      6. Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Wolters Kluwer. 979-82, 2019
                                                                                      7. Nagaraj UD et al: Spinal imaging findings of open spinal dysraphisms on fetal and postnatal MRI. AJNR Am J Neuroradiol. 39(10):1947-52, 2018
                                                                                      8. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                                                                      9. Caldarelli M et al: Recurrent tethered cord: radiological investigation and management. Childs Nerv Syst. 29(9):1601-9, 2013
                                                                                      10. Messing-Jünger M et al: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 29(9):1553-62, 2013
                                                                                      11. Sweeney KJ et al: Spinal level of myelomeningocele lesion as a contributing factor in posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia. J Neurosurg Pediatr. 11(2):154-9, 2013
                                                                                      12. Jernigan SC et al: Risk factors of sudden death in young adult patients with myelomeningocele. J Neurosurg Pediatr. 9(2):149-55, 2012
                                                                                      13. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                                                                      14. Chao TT et al: Fetal spine findings on MRI and associated outcomes in children with open neural tube defects. AJR Am J Roentgenol. 197(5):W956-61, 2011
                                                                                      15. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                                                                      16. Rufener SL et al: Congenital spine and spinal cord malformations--pictorial review. AJR Am J Roentgenol. 194(3 Suppl):S26-37, 2010
                                                                                      17. Hirose S et al: Fetal surgery for myelomeningocele. Clin Perinatol. 36(2):431-8, xi, 2009
                                                                                      18. Hirose S et al: Fetal surgery for myelomeningocele: panacea or peril? World J Surg. 27(1):87-94, 2003
                                                                                      19. Johnson MP et al: Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol. 189(2):482-7, 2003
                                                                                      20. Ertl-Wagner B et al: Fetal magnetic resonance imaging: indications, technique, anatomical considerations and a review of fetal abnormalities. Eur Radiol. 12(8):1931-40, 2002
                                                                                      21. Rintoul NE et al: A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics. 109(3):409-13, 2002
                                                                                      22. Trivedi J et al: Clinical and radiographic predictors of scoliosis in patients with myelomeningocele. J Bone Joint Surg Am. 84-A(8):1389-94, 2002
                                                                                      23. Bowman RM et al: Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 34(3):114-20, 2001
                                                                                      24. 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
                                                                                      25. Unsinn KM et al: US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases. Radiographics. 20(4):923-38, 2000