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Chiari 2
Usha D. Nagaraj, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Chiari type II, Arnold-Chiari malformation
            • Definitions

              • Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of myelomeningocele (MMC) or myelocele/myeloschisis
                • Vast majority of patients with MMC have CM2
                • Few case reports of CM2 without MMC have other congenital CSF leaks (e.g., patent craniopharyngeal duct)

            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. Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 1-8, 2015
                                            2. Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
                                            3. 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
                                            4. Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 2013
                                            5. 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
                                            6. 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
                                            7. Barkovich AJ et al: Pediatric Neuroimaging, 5th edition. Philadelphia: Lippincott Williams & Wilkins. 497-501, 2012
                                            8. 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
                                            9. 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
                                            10. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                            11. 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
                                            12. 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
                                            13. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                            14. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                            15. Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
                                            16. 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

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Chiari type II, Arnold-Chiari malformation
                                                      • Definitions

                                                        • Chiari 2 malformation (CM2): Constellation of characteristic intracranial anomalies, mainly hindbrain herniation, due to open spinal dysraphism of myelomeningocele (MMC) or myelocele/myeloschisis
                                                          • Vast majority of patients with MMC have CM2
                                                          • Few case reports of CM2 without MMC have other congenital CSF leaks (e.g., patent craniopharyngeal duct)

                                                      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. Tulipan N et al: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 1-8, 2015
                                                                                      2. Kobraei EM et al: A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 30(9):1535-41, 2014
                                                                                      3. 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
                                                                                      4. Akbari SH et al: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 29(7):1143-54, 2013
                                                                                      5. 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
                                                                                      6. 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
                                                                                      7. Barkovich AJ et al: Pediatric Neuroimaging, 5th edition. Philadelphia: Lippincott Williams & Wilkins. 497-501, 2012
                                                                                      8. 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
                                                                                      9. 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
                                                                                      10. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011
                                                                                      11. 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
                                                                                      12. 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
                                                                                      13. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                                                                      14. McLone DG et al: The Chiari II malformation: cause and impact. Childs Nerv Syst. 19(7-8):540-50, 2003
                                                                                      15. Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
                                                                                      16. McLone DG et al: The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 15(1):1-12, 1989