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Pediatrics
Diagnosis
Spine
Congenital and Genetic Disorders
Congenital
Chiari Disorders
Chiari 2 Malformation
Chiari 2 Malformation
Usha D. Nagaraj, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Chiari II malformation, Chiari II, Chiari malformation

              • Definitions

                • Constellation of intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism [either myelomeningocele (MMC) or myelocele]

              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

                                            • 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. Talamonti G et al: Surgical management of Chiari malformation type II. Childs Nerv Syst. 36(8):1621-34, 2020
                                                3. Miller JL et al: Spinal dysraphia, Chiari 2 malformation, unified theory, and advances in fetoscopic repair. Neuroimaging Clin N Am. 29(3):357-66, 2019
                                                4. Kim I et al: Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry. J Neurosurg Pediatr. 22(6):652-8, 2018
                                                5. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                                6. Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 16(6):613-20, 2015
                                                7. Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
                                                8. Kshettry VR et al: Myelomeningocele: surgical trends and predictors of outcome in the United States, 1988-2010. J Neurosurg Pediatr. 13(6):666-78, 2014
                                                9. Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 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. Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Lippincott Williams & Wilkins. 554-560, 2019
                                                13. Citton V et al: Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis? J Child Neurol. 27(4):536-9, 2012
                                                14. Geerdink N et al: Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1. Childs Nerv Syst. 28(7):977-85, 2012
                                                15. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                                16. Juranek J et al: The cerebellum in children with spina bifida and Chiari II malformation: quantitative volumetrics by region. Cerebellum. 9(2):240-8, 2010
                                                17. Morota N et al: Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele. J Neurosurg Pediatr. 2(3):188-93, 2008
                                                18. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                                19. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                                20. Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
                                                21. McLone DG et al: The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 15(1):1-12, 1989
                                                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

                                                              • Chiari II malformation, Chiari II, Chiari malformation

                                                            • Definitions

                                                              • Constellation of intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism [either myelomeningocele (MMC) or myelocele]

                                                            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

                                                                                          • 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. Talamonti G et al: Surgical management of Chiari malformation type II. Childs Nerv Syst. 36(8):1621-34, 2020
                                                                                              3. Miller JL et al: Spinal dysraphia, Chiari 2 malformation, unified theory, and advances in fetoscopic repair. Neuroimaging Clin N Am. 29(3):357-66, 2019
                                                                                              4. Kim I et al: Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry. J Neurosurg Pediatr. 22(6):652-8, 2018
                                                                                              5. Nagaraj UD et al: Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 38(5):1031-6, 2017
                                                                                              6. Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 16(6):613-20, 2015
                                                                                              7. Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
                                                                                              8. Kshettry VR et al: Myelomeningocele: surgical trends and predictors of outcome in the United States, 1988-2010. J Neurosurg Pediatr. 13(6):666-78, 2014
                                                                                              9. Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 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. Barkovich AJ et al: Pediatric Neuroimaging. 6th ed. Lippincott Williams & Wilkins. 554-560, 2019
                                                                                              13. Citton V et al: Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis? J Child Neurol. 27(4):536-9, 2012
                                                                                              14. Geerdink N et al: Essential features of Chiari II malformation in MR imaging: an interobserver reliability study--part 1. Childs Nerv Syst. 28(7):977-85, 2012
                                                                                              15. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                                                                              16. Juranek J et al: The cerebellum in children with spina bifida and Chiari II malformation: quantitative volumetrics by region. Cerebellum. 9(2):240-8, 2010
                                                                                              17. Morota N et al: Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele. J Neurosurg Pediatr. 2(3):188-93, 2008
                                                                                              18. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                                                                              19. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                                                                              20. Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
                                                                                              21. McLone DG et al: The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 15(1):1-12, 1989